CLPB

Functionⁱ

May function as a regulatory ATPase and be related to secretion/protein trafficking process. Involved in mitochondrial-mediated antiviral innate immunity, activates RIG-I-mediated signal transduction and production of IFNB1 and proinflammatory cytokine IL6 (PubMed:31522117).1 Publication

Catalytic activityⁱ

ATP
Search proteins in UniProtKB for this molecule.
Search chemical reactions in Rhea for this molecule.
See the description of this molecule in ChEBI.
+
H₂O
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Search chemical reactions in Rhea for this molecule.
See the description of this molecule in ChEBI.
=
ADP
Search proteins in UniProtKB for this molecule.
Search chemical reactions in Rhea for this molecule.
See the description of this molecule in ChEBI.
+
H⁺
Search proteins in UniProtKB for this molecule.
Search chemical reactions in Rhea for this molecule.
See the description of this molecule in ChEBI.
+
phosphate
Search proteins in UniProtKB for this molecule.
Search chemical reactions in Rhea for this molecule.
See the description of this molecule in ChEBI.
1 Publication
Manual assertion based on experiment inⁱ
- Ref.10
  "CLPB mutations cause 3-methylglutaconic aciduria, progressive brain atrophy, intellectual disability, congenital neutropenia, cataracts, movement disorder."
  Wortmann S.B., Zietkiewicz S., Kousi M., Szklarczyk R., Haack T.B., Gersting S.W., Muntau A.C., Rakovic A., Renkema G.H., Rodenburg R.J., Strom T.M., Meitinger T., Rubio-Gozalbo M.E., Chrusciel E., Distelmaier F., Golzio C., Jansen J.H., van Karnebeek C.
  Wevers R.A.
  Am. J. Hum. Genet. 96:245-257(2015) [PubMed] [Europe PMC] [Abstract]
  Cited for: VARIANTS MGCA7 CYS-272; GLY-408; ILE-411; 435-ASP-PRO-436 DELINS ASP-PRO; ARG-486; LYS-501; CYS-567; VAL-591; CYS-617; VAL-646 AND ASN-682, CATALYTIC ACTIVITY, SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
Source:
Rhea
Search for this reaction in UniProtKB.
See the description of this reaction in Rhea.
. Show »« Hide
ATP
+
H₂O
=
ADP
+
H⁺
+
phosphate
zoom

Regions

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Nucleotide bindingⁱ	381 – 388	ATPSequence analysis		8

GO - Molecular functionⁱ

ATPase activity Source: UniProtKB-EC
ATP binding Source: UniProtKB-KW

Complete GO annotation on QuickGO ...

GO - Biological processⁱ

Complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Hydrolase
Ligand	ATP-binding, Nucleotide-binding

Enzyme and pathway databases

PathwayCommonsⁱ	Q9H078

PathwayCommonsⁱ

Q9H078

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Caseinolytic peptidase B protein homolog (EC:3.6.1.-1 Publication) Alternative name(s): Suppressor of potassium transport defect 3
Gene namesⁱ	Name:CLPB Synonyms:HSP78, SKD3
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 11

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000162129.12
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:30664, CLPB
MIMⁱ	616254, gene
neXtProtⁱ	NX_Q9H078

Keywords - Cellular componentⁱ

Mitochondrion

Pathology & Biotechⁱ

Involvement in diseaseⁱ

3-methylglutaconic aciduria 7 (MGCA7)2 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionAn autosomal recessive inborn error of metabolism with a highly variable phenotype. Primary disease symptoms are increased levels of 3-methylglutaconic acid, neurologic deterioration and neutropenia. Other common features include progressive encephalopathy, movement abnormalities, delayed psychomotor development, cataracts, seizures, and recurrent infections.

Related information in OMIM

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_073397	268	T → M in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs200032855Ensembl.	1
Natural variantⁱVAR_073398	272	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs777313457Ensembl.	1
Natural variantⁱVAR_073399	408	R → G in MGCA7; reduced ATPase activity in vitro. 1 PublicationCorresponds to variant dbSNP:rs144078282Ensembl.	1
Natural variantⁱVAR_073400	411	M → I in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs786205137Ensembl.	1
Natural variantⁱVAR_073401	435 – 436	EG → DP in MGCA7. 1 Publication	2
Natural variantⁱVAR_073402	486	C → R in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs886041118Ensembl.	1
Natural variantⁱVAR_073403	501	E → K in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs748915609Ensembl.	1
Natural variantⁱVAR_073404	567	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs150857620Ensembl.	1
Natural variantⁱVAR_073405	591	A → V in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs748010262Ensembl.	1
Natural variantⁱVAR_073406	617	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs786205138Ensembl.	1
Natural variantⁱVAR_073407	646	G → V in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs759500860Ensembl.	1
Natural variantⁱVAR_073408	682	I → N in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs886041120Ensembl.	1

Keywords - Diseaseⁱ

Cataract, Disease mutation, Epilepsy

Organism-specific databases

DisGeNET More...DisGeNETⁱ	81570
GeneReviewsⁱ	CLPB
MalaCards human disease database More...MalaCardsⁱ	CLPB
MIMⁱ	616271, phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000162129
Orphanetⁱ	445038, 3-methylglutaconic aciduria type 7
PharmGKBⁱ	PA142672092

Miscellaneous databases

Pharosⁱ	Q9H078, Tbio

Pharosⁱ

Q9H078, Tbio

Polymorphism and mutation databases

BioMutaⁱ	CLPB
DMDMⁱ	25009267

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 36	MitochondrionSequence analysisAdd BLAST		36
ChainⁱPRO_0000191239	37 – 707	Caseinolytic peptidase B protein homologAdd BLAST		671

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Modified residueⁱ	589	N6-acetyllysineCombined sources		1

Keywords - PTMⁱ

Acetylation

Proteomic databases

EPDⁱ	Q9H078
jPOSTⁱ	Q9H078
MassIVEⁱ	Q9H078
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q9H078
PaxDbⁱ	Q9H078
PeptideAtlas More...PeptideAtlasⁱ	Q9H078
PRIDEⁱ	Q9H078
ProteomicsDBⁱ	18882 29984 5473 80212 [Q9H078-1] 80213 [Q9H078-2]

PTM databases

iPTMnetⁱ	Q9H078
MetOSiteⁱ	Q9H078
PhosphoSitePlusⁱ	Q9H078
SwissPalmⁱ	Q9H078

Expressionⁱ

Tissue specificityⁱ

Widely expressed (at protein level) (PubMed:25597511). Expressed in fetal, as well as in adult tissues, with highest levels in adult brain, including thalamus, hippocampus, occipital cortex and parietal cortex. Low expression in granulocytes (PubMed:25597510).2 Publications

Gene expression databases

Bgeeⁱ	ENSG00000162129, Expressed in right testis and 182 other tissues
ExpressionAtlasⁱ	Q9H078, baseline and differential
Genevisibleⁱ	Q9H078, HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	ENSG00000162129, Tissue enriched (testis)

HPAⁱ

ENSG00000162129, Tissue enriched (testis)

Interactionⁱ

Subunit structureⁱ

Interacts with PHB and PHB2 (PubMed:31522117).

Interacts with MAVS; the interaction is enhanced by Sendai virus infection (PubMed:31522117).

1 Publication

Binary interactionsⁱ

Q9H078

With	#Exp.	IntAct
CEP70 [Q8NHQ1]	3	EBI-2107221,EBI-739624

Protein-protein interaction databases

BioGRIDⁱ	123531, 77 interactors
IntActⁱ	Q9H078, 65 interactors
Molecular INTeraction database More...MINTⁱ	Q9H078
STRINGⁱ	9606.ENSP00000294053

Miscellaneous databases

RNActⁱ	Q9H078, protein

RNActⁱ

Q9H078, protein

Structureⁱ

3D structure databases

SMRⁱ	Q9H078
ModBaseⁱ	Search...

Family & Domainsⁱ

Domains and Repeats

Feature key	Position(s)	DescriptionActions	Length
Repeatⁱ	133 – 162	ANK 1Add BLAST	30
Repeatⁱ	166 – 195	ANK 2Add BLAST	30
Repeatⁱ	265 – 295	ANK 3Add BLAST	31
Repeatⁱ	298 – 327	ANK 4Add BLAST	30

Sequence similaritiesⁱ

Belongs to the ClpA/ClpB family.Curated

Keywords - Domainⁱ

ANK repeat, Repeat, Transit peptide

Phylogenomic databases

eggNOGⁱ	KOG1051, Eukaryota
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000012961
HOGENOMⁱ	CLU_005070_9_3_1
InParanoidⁱ	Q9H078
KEGG Orthology (KO) More...KOⁱ	K03695
PhylomeDBⁱ	Q9H078
TreeFamⁱ	TF328654

Family and domain databases

Gene3Dⁱ	1.25.40.20, 2 hits
InterProⁱ	View protein in InterPro IPR003593, AAA+_ATPase IPR002110, Ankyrin_rpt IPR020683, Ankyrin_rpt-contain_dom IPR036770, Ankyrin_rpt-contain_sf IPR003959, ATPase_AAA_core IPR019489, Clp_ATPase_C IPR001270, ClpA/B IPR027417, P-loop_NTPase
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF07724, AAA_2, 1 hit PF12796, Ank_2, 2 hits PF10431, ClpB_D2-small, 1 hit
PRINTSⁱ	PR00300, CLPPROTEASEA
SMARTⁱ	View protein in SMART SM00382, AAA, 1 hit SM00248, ANK, 3 hits SM01086, ClpB_D2-small, 1 hit
SUPFAMⁱ	SSF48403, SSF48403, 1 hit SSF52540, SSF52540, 1 hit
PROSITEⁱ	View protein in PROSITE PS50297, ANK_REP_REGION, 1 hit PS50088, ANK_REPEAT, 3 hits

Sequences (5+)ⁱ

Sequence statusⁱ: Complete.

Sequence processingⁱ: The displayed sequence is further processed into a mature form.

This entry describes 5 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 5 described isoforms and 11 potential isoforms that are computationally mapped.Show all Align All

Isoform 1 (identifier: Q9H078-1) [UniParc]FASTA Add to basket

This isoform has been chosen as the canonicalⁱ sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

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        10         20         30         40         50
MLGSLVLRRK ALAPRLLLRL LRSPTLRGHG GASGRNVTTG SLGEPQWLRV 
        60         70         80         90        100
ATGGRPGTSP ALFSGRGAAT GGRQGGRFDT KCLAAATWGR LPGPEETLPG 
       110        120        130        140        150
QDSWNGVPSR AGLGMCALAA ALVVHCYSKS PSNKDAALLE AARANNMQEV 
       160        170        180        190        200
SRLLSEGADV NAKHRLGWTA LMVAAINRNN SVVQVLLAAG ADPNLGDDFS 
       210        220        230        240        250
SVYKTAKEQG IHSLEDGGQD GASRHITNQW TSALEFRRWL GLPAGVLITR 
       260        270        280        290        300
EDDFNNRLNN RASFKGCTAL HYAVLADDYR TVKELLDGGA NPLQRNEMGH 
       310        320        330        340        350
TPLDYAREGE VMKLLRTSEA KYQEKQRKRE AEERRRFPLE QRLKEHIIGQ 
       360        370        380        390        400
ESAIATVGAA IRRKENGWYD EEHPLVFLFL GSSGIGKTEL AKQTAKYMHK 
       410        420        430        440        450
DAKKGFIRLD MSEFQERHEV AKFIGSPPGY VGHEEGGQLT KKLKQCPNAV 
       460        470        480        490        500
VLFDEVDKAH PDVLTIMLQL FDEGRLTDGK GKTIDCKDAI FIMTSNVASD 
       510        520        530        540        550
EIAQHALQLR QEALEMSRNR IAENLGDVQI SDKITISKNF KENVIRPILK 
       560        570        580        590        600
AHFRRDEFLG RINEIVYFLP FCHSELIQLV NKELNFWAKR AKQRHNITLL 
       610        620        630        640        650
WDREVADVLV DGYNVHYGAR SIKHEVERRV VNQLAAAYEQ DLLPGGCTLR 
       660        670        680        690        700
ITVEDSDKQL LKSPELPSPQ AEKRLPKLRL EIIDKDSKTR RLDIRAPLHP 

EKVCNTI

Length:707

Mass (Da):78,729

Last modified:March 1, 2001 - v1

Checksum:ⁱ0E0F2A244CA20635

GO

Isoform 2 (identifier: Q9H078-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
216-245: Missing.

« Hide

        10         20         30         40         50
MLGSLVLRRK ALAPRLLLRL LRSPTLRGHG GASGRNVTTG SLGEPQWLRV 
        60         70         80         90        100
ATGGRPGTSP ALFSGRGAAT GGRQGGRFDT KCLAAATWGR LPGPEETLPG 
       110        120        130        140        150
QDSWNGVPSR AGLGMCALAA ALVVHCYSKS PSNKDAALLE AARANNMQEV 
       160        170        180        190        200
SRLLSEGADV NAKHRLGWTA LMVAAINRNN SVVQVLLAAG ADPNLGDDFS 
       210        220        230        240        250
SVYKTAKEQG IHSLEVLITR EDDFNNRLNN RASFKGCTAL HYAVLADDYR 
       260        270        280        290        300
TVKELLDGGA NPLQRNEMGH TPLDYAREGE VMKLLRTSEA KYQEKQRKRE 
       310        320        330        340        350
AEERRRFPLE QRLKEHIIGQ ESAIATVGAA IRRKENGWYD EEHPLVFLFL 
       360        370        380        390        400
GSSGIGKTEL AKQTAKYMHK DAKKGFIRLD MSEFQERHEV AKFIGSPPGY 
       410        420        430        440        450
VGHEEGGQLT KKLKQCPNAV VLFDEVDKAH PDVLTIMLQL FDEGRLTDGK 
       460        470        480        490        500
GKTIDCKDAI FIMTSNVASD EIAQHALQLR QEALEMSRNR IAENLGDVQI 
       510        520        530        540        550
SDKITISKNF KENVIRPILK AHFRRDEFLG RINEIVYFLP FCHSELIQLV 
       560        570        580        590        600
NKELNFWAKR AKQRHNITLL WDREVADVLV DGYNVHYGAR SIKHEVERRV 
       610        620        630        640        650
VNQLAAAYEQ DLLPGGCTLR ITVEDSDKQL LKSPELPSPQ AEKRLPKLRL 
       660        670 
EIIDKDSKTR RLDIRAPLHP EKVCNTI

Show »

Length:677

Mass (Da):75,449

Checksum:ⁱC03F18C94337B9E4

GO

Isoform 3 (identifier: Q9H078-3) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
152-180: Missing.
216-245: Missing.

« Hide

        10         20         30         40         50
MLGSLVLRRK ALAPRLLLRL LRSPTLRGHG GASGRNVTTG SLGEPQWLRV 
        60         70         80         90        100
ATGGRPGTSP ALFSGRGAAT GGRQGGRFDT KCLAAATWGR LPGPEETLPG 
       110        120        130        140        150
QDSWNGVPSR AGLGMCALAA ALVVHCYSKS PSNKDAALLE AARANNMQEV 
       160        170        180        190        200
SSVVQVLLAA GADPNLGDDF SSVYKTAKEQ GIHSLEVLIT REDDFNNRLN 
       210        220        230        240        250
NRASFKGCTA LHYAVLADDY RTVKELLDGG ANPLQRNEMG HTPLDYAREG 
       260        270        280        290        300
EVMKLLRTSE AKYQEKQRKR EAEERRRFPL EQRLKEHIIG QESAIATVGA 
       310        320        330        340        350
AIRRKENGWY DEEHPLVFLF LGSSGIGKTE LAKQTAKYMH KDAKKGFIRL 
       360        370        380        390        400
DMSEFQERHE VAKFIGSPPG YVGHEEGGQL TKKLKQCPNA VVLFDEVDKA 
       410        420        430        440        450
HPDVLTIMLQ LFDEGRLTDG KGKTIDCKDA IFIMTSNVAS DEIAQHALQL 
       460        470        480        490        500
RQEALEMSRN RIAENLGDVQ ISDKITISKN FKENVIRPIL KAHFRRDEFL 
       510        520        530        540        550
GRINEIVYFL PFCHSELIQL VNKELNFWAK RAKQRHNITL LWDREVADVL 
       560        570        580        590        600
VDGYNVHYGA RSIKHEVERR VVNQLAAAYE QDLLPGGCTL RITVEDSDKQ 
       610        620        630        640 
LLKSPELPSP QAEKRLPKLR LEIIDKDSKT RRLDIRAPLH PEKVCNTI

Show »

Length:648

Mass (Da):72,276

Checksum:ⁱ586F3100E877B4D4

GO

Isoform 4 (identifier: Q9H078-4) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
1-151: MLGSLVLRRK...ARANNMQEVS → MPRGCHLGTP...TKISSSEWPL

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        10         20         30         40         50
MPRGCHLGTP SWSRRNTPRT GQLERGPQQG RTGHVRPGRS AGGSLLQQES 
        60         70         80         90        100
VQQGCSPVGS CPCQQYARSQ QPQETAKNDA QSRSWAGLNA GVSLKNTKIS 
       110        120        130        140        150
SSEWPLRLLS EGADVNAKHR LGWTALMVAA INRNNSVVQV LLAAGADPNL 
       160        170        180        190        200
GDDFSSVYKT AKEQGIHSLE DGGQDGASRH ITNQWTSALE FRRWLGLPAG 
       210        220        230        240        250
VLITREDDFN NRLNNRASFK GCTALHYAVL ADDYRTVKEL LDGGANPLQR 
       260        270        280        290        300
NEMGHTPLDY AREGEVMKLL RTSEAKYQEK QRKREAEERR RFPLEQRLKE 
       310        320        330        340        350
HIIGQESAIA TVGAAIRRKE NGWYDEEHPL VFLFLGSSGI GKTELAKQTA 
       360        370        380        390        400
KYMHKDAKKG FIRLDMSEFQ ERHEVAKFIG SPPGYVGHEE GGQLTKKLKQ 
       410        420        430        440        450
CPNAVVLFDE VDKAHPDVLT IMLQLFDEGR LTDGKGKTID CKDAIFIMTS 
       460        470        480        490        500
NVASDEIAQH ALQLRQEALE MSRNRIAENL GDVQISDKIT ISKNFKENVI 
       510        520        530        540        550
RPILKAHFRR DEFLGRINEI VYFLPFCHSE LIQLVNKELN FWAKRAKQRH 
       560        570        580        590        600
NITLLWDREV ADVLVDGYNV HYGARSIKHE VERRVVNQLA AAYEQDLLPG 
       610        620        630        640        650
GCTLRITVED SDKQLLKSPE LPSPQAEKRL PKLRLEIIDK DSKTRRLDIR 
       660 
APLHPEKVCN TI

Show »

Length:662

Mass (Da):74,543

Checksum:ⁱF9DE15704672A495

GO

Isoform 5 (identifier: Q9H078-5) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
1-171: Missing.
216-245: Missing.

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        10         20         30         40         50
MVAAINRNNS VVQVLLAAGA DPNLGDDFSS VYKTAKEQGI HSLEVLITRE 
        60         70         80         90        100
DDFNNRLNNR ASFKGCTALH YAVLADDYRT VKELLDGGAN PLQRNEMGHT 
       110        120        130        140        150
PLDYAREGEV MKLLRTSEAK YQEKQRKREA EERRRFPLEQ RLKEHIIGQE 
       160        170        180        190        200
SAIATVGAAI RRKENGWYDE EHPLVFLFLG SSGIGKTELA KQTAKYMHKD 
       210        220        230        240        250
AKKGFIRLDM SEFQERHEVA KFIGSPPGYV GHEEGGQLTK KLKQCPNAVV 
       260        270        280        290        300
LFDEVDKAHP DVLTIMLQLF DEGRLTDGKG KTIDCKDAIF IMTSNVASDE 
       310        320        330        340        350
IAQHALQLRQ EALEMSRNRI AENLGDVQIS DKITISKNFK ENVIRPILKA 
       360        370        380        390        400
HFRRDEFLGR INEIVYFLPF CHSELIQLVN KELNFWAKRA KQRHNITLLW 
       410        420        430        440        450
DREVADVLVD GYNVHYGARS IKHEVERRVV NQLAAAYEQD LLPGGCTLRI 
       460        470        480        490        500
TVEDSDKQLL KSPELPSPQA EKRLPKLRLE IIDKDSKTRR LDIRAPLHPE 

KVCNTI

Show »

Length:506

Mass (Da):57,664

Checksum:ⁱ55E4A602D8B4F44A

GO

Computationally mapped potential isoform sequencesⁱ

There are 11 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

	Entry	Entry name	Protein names	Gene names	Length	Annotation

	H0YGM0	H0YGM0_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	712	Annotation score:
	A0A2R8Y602	A0A2R8Y602_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	382	Annotation score:
	A0A2U3TZY2	A0A2U3TZY2_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	692	Annotation score:
	A0A2R8YDH5	A0A2R8YDH5_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	141	Annotation score:
	F5H392	F5H392_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	149	Annotation score:
	F5H7A5	F5H7A5_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	152	Annotation score:
	A0A2R8Y6R5	A0A2R8Y6R5_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	327	Annotation score:
	F5GX99	F5GX99_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	149	Annotation score:
	F6SS08	F6SS08_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	75	Annotation score:
	H0YG50	H0YG50_HUMAN	Caseinolytic peptidase B protein ho... Caseinolytic peptidase B protein homolog	CLPB	44	Annotation score:
There is more potential isoformShow all

Experimental Info

Feature key	Position(s)	DescriptionActions	Length
Sequence conflictⁱ	413	E → K in BAG63459 (PubMed:14702039).Curated	1
Sequence conflictⁱ	563	N → S in BAG63409 (PubMed:14702039).Curated	1
Sequence conflictⁱ	650	R → C in BAG63459 (PubMed:14702039).Curated	1

Natural variant

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_073397	268	T → M in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs200032855Ensembl.	1
Natural variantⁱVAR_073398	272	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs777313457Ensembl.	1
Natural variantⁱVAR_048740	295	R → T. Corresponds to variant dbSNP:rs7938203Ensembl.	1
Natural variantⁱVAR_073399	408	R → G in MGCA7; reduced ATPase activity in vitro. 1 PublicationCorresponds to variant dbSNP:rs144078282Ensembl.	1
Natural variantⁱVAR_073400	411	M → I in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs786205137Ensembl.	1
Natural variantⁱVAR_073401	435 – 436	EG → DP in MGCA7. 1 Publication	2
Natural variantⁱVAR_073402	486	C → R in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs886041118Ensembl.	1
Natural variantⁱVAR_073403	501	E → K in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs748915609Ensembl.	1
Natural variantⁱVAR_073404	567	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs150857620Ensembl.	1
Natural variantⁱVAR_073405	591	A → V in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs748010262Ensembl.	1
Natural variantⁱVAR_073406	617	Y → C in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs786205138Ensembl.	1
Natural variantⁱVAR_073407	646	G → V in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs759500860Ensembl.	1
Natural variantⁱVAR_073408	682	I → N in MGCA7. 1 PublicationCorresponds to variant dbSNP:rs886041120Ensembl.	1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Length
Alternative sequenceⁱVSP_057397	1 – 171	Missing in isoform 5. 1 PublicationAdd BLAST	171
Alternative sequenceⁱVSP_044725	1 – 151	MLGSL…MQEVS → MPRGCHLGTPSWSRRNTPRT GQLERGPQQGRTGHVRPGRS AGGSLLQQESVQQGCSPVGS CPCQQYARSQQPQETAKNDA QSRSWAGLNAGVSLKNTKIS SSEWPL in isoform 4. 1 PublicationAdd BLAST	151
Alternative sequenceⁱVSP_044726	152 – 180	Missing in isoform 3. 1 PublicationAdd BLAST	29
Alternative sequenceⁱVSP_001106	216 – 245	Missing in isoform 2, isoform 3 and isoform 5. 1 PublicationAdd BLAST	30

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AL136909 mRNA Translation: CAB66843.1 AL834484 mRNA Translation: CAD39142.1 AK023214 mRNA Translation: BAB14467.1 AK302006 mRNA Translation: BAG63409.1 AK302069 mRNA Translation: BAG63459.1 AK302158 mRNA Translation: BAG63526.1 AP000593 Genomic DNA No translation available. AP002892 Genomic DNA No translation available. AP003785 Genomic DNA No translation available. BC006404 mRNA Translation: AAH06404.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS58152.1 [Q9H078-4] CCDS58153.1 [Q9H078-3] CCDS58154.1 [Q9H078-2] CCDS8215.1 [Q9H078-1]
NCBI Reference Sequences More...RefSeqⁱ	NP_001245321.1, NM_001258392.2 [Q9H078-2] NP_001245322.1, NM_001258393.2 [Q9H078-3] NP_001245323.1, NM_001258394.2 [Q9H078-4] NP_110440.1, NM_030813.5 [Q9H078-1]

Genome annotation databases

Ensemblⁱ	ENST00000294053; ENSP00000294053; ENSG00000162129 [Q9H078-1] ENST00000340729; ENSP00000340385; ENSG00000162129 [Q9H078-3] ENST00000437826; ENSP00000407296; ENSG00000162129 [Q9H078-4] ENST00000538039; ENSP00000441518; ENSG00000162129 [Q9H078-2]
GeneIDⁱ	81570
KEGGⁱ	hsa:81570
UCSC genome browser More...UCSCⁱ	uc001osj.5, human [Q9H078-1] uc010rqz.3, human

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q9H078	ClpB caseinolytic peptidase B homolog (E. coli), isoform CRA_a		HUMAN		707	UniRef100_Q9H078
CLPB isoform 11 (Fragment)		PANTR		149
Caseinolytic peptidase B protein homolog (Fragment)		HUMAN		149
Q9H078-4	Caseinolytic peptidase B protein homolog		PANTR		410	UniRef100_G2HGX8
Caseinolytic peptidase B protein homolog (Fragment)		HUMAN		712
Q9H078-5	CLPB isoform 5		PANTR		677	UniRef100_K7DLH6
Caseinolytic peptidase B protein homolog (Fragment)		HUMAN		382
CLPB isoform 10 (Fragment)		PANTR		153
CLPB isoform 3		PANTR		506
Caseinolytic peptidase B protein homolog (Fragment)		HUMAN		152

Protein	Similar proteins		Species	Score	Length	Source
Q9H078	ClpB caseinolytic peptidase B homolog (E. coli), isoform CRA_a		HUMAN		707	UniRef90_Q9H078
CLPB isoform 11 (Fragment)		PANTR		149
ANK_REP_REGION domain-containing protein		SHEEP		807
ClpB homolog, mitochondrial AAA ATPase chaperonin		HORSE		720
ClpB homolog, mitochondrial AAA ATPase chaperonin		SURSU		714
+264
Q9H078-4	ANK_REP_REGION domain-containing protein		PAPAN		704	UniRef90_A0A2I3M1E8
ANK_REP_REGION domain-containing protein		CALJA		712
ANK_REP_REGION domain-containing protein		MANLE		712
Caseinolytic peptidase B protein homolog (Fragment)		MACMU		461
ANK_REP_REGION domain-containing protein		COLAP		461
+25

Protein	Similar proteins		Species	Score	Length	Source
Q9H078	ClpB caseinolytic peptidase B homolog (E. coli), isoform CRA_a		HUMAN		707	UniRef50_Q9H078
CLPB isoform 11 (Fragment)		PANTR		149
ANK_REP_REGION domain-containing protein		SHEEP		807
ClpB homolog, mitochondrial AAA ATPase chaperonin		HORSE		720
ClpB homolog, mitochondrial AAA ATPase chaperonin		SURSU		714
+317
Q9H078-4	Caseinolytic peptidase B protein homolog		MOUSE		677	UniRef50_Q60649
Caseinolytic peptidase B protein homolog		RAT		677
Caseinolytic peptidase B protein homolog		BOVIN		677
caseinolytic peptidase B protein homolog		MESAU		680
ANK_REP_REGION domain-containing protein		PROCO		677
+272

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AL136909 mRNA Translation: CAB66843.1 AL834484 mRNA Translation: CAD39142.1 AK023214 mRNA Translation: BAB14467.1 AK302006 mRNA Translation: BAG63409.1 AK302069 mRNA Translation: BAG63459.1 AK302158 mRNA Translation: BAG63526.1 AP000593 Genomic DNA No translation available. AP002892 Genomic DNA No translation available. AP003785 Genomic DNA No translation available. BC006404 mRNA Translation: AAH06404.1
CCDSⁱ	CCDS58152.1 [Q9H078-4] CCDS58153.1 [Q9H078-3] CCDS58154.1 [Q9H078-2] CCDS8215.1 [Q9H078-1]
RefSeqⁱ	NP_001245321.1, NM_001258392.2 [Q9H078-2] NP_001245322.1, NM_001258393.2 [Q9H078-3] NP_001245323.1, NM_001258394.2 [Q9H078-4] NP_110440.1, NM_030813.5 [Q9H078-1]

3D structure databases

SMRⁱ	Q9H078
ModBaseⁱ	Search...

Protein-protein interaction databases

BioGRIDⁱ	123531, 77 interactors
IntActⁱ	Q9H078, 65 interactors
MINTⁱ	Q9H078
STRINGⁱ	9606.ENSP00000294053

PTM databases

iPTMnetⁱ	Q9H078
MetOSiteⁱ	Q9H078
PhosphoSitePlusⁱ	Q9H078
SwissPalmⁱ	Q9H078

Polymorphism and mutation databases

BioMutaⁱ	CLPB
DMDMⁱ	25009267

Proteomic databases

EPDⁱ	Q9H078
jPOSTⁱ	Q9H078
MassIVEⁱ	Q9H078
MaxQBⁱ	Q9H078
PaxDbⁱ	Q9H078
PeptideAtlasⁱ	Q9H078
PRIDEⁱ	Q9H078
ProteomicsDBⁱ	18882 29984 5473 80212 [Q9H078-1] 80213 [Q9H078-2]

Protocols and materials databases

Antibodypediaⁱ	30838, 185 antibodies
The DNASU plasmid repository More...DNASUⁱ	81570

Genome annotation databases

Ensemblⁱ	ENST00000294053; ENSP00000294053; ENSG00000162129 [Q9H078-1] ENST00000340729; ENSP00000340385; ENSG00000162129 [Q9H078-3] ENST00000437826; ENSP00000407296; ENSG00000162129 [Q9H078-4] ENST00000538039; ENSP00000441518; ENSG00000162129 [Q9H078-2]
GeneIDⁱ	81570
KEGGⁱ	hsa:81570
UCSCⁱ	uc001osj.5, human [Q9H078-1] uc010rqz.3, human

Organism-specific databases

CTDⁱ	81570
DisGeNETⁱ	81570
EuPathDBⁱ	HostDB:ENSG00000162129.12
GeneCardsⁱ	CLPB
GeneReviewsⁱ	CLPB
HGNCⁱ	HGNC:30664, CLPB
HPAⁱ	ENSG00000162129, Tissue enriched (testis)
MalaCardsⁱ	CLPB
MIMⁱ	616254, gene 616271, phenotype
neXtProtⁱ	NX_Q9H078
OpenTargetsⁱ	ENSG00000162129
Orphanetⁱ	445038, 3-methylglutaconic aciduria type 7
PharmGKBⁱ	PA142672092
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG1051, Eukaryota
GeneTreeⁱ	ENSGT00390000012961
HOGENOMⁱ	CLU_005070_9_3_1
InParanoidⁱ	Q9H078
KOⁱ	K03695
PhylomeDBⁱ	Q9H078
TreeFamⁱ	TF328654

Enzyme and pathway databases

PathwayCommonsⁱ	Q9H078

PathwayCommonsⁱ

Q9H078

Miscellaneous databases

BioGRID-ORCSⁱ	81570, 91 hits in 872 CRISPR screens
ChiTaRSⁱ	CLPB, human
GeneWikiⁱ	CLPB
GenomeRNAiⁱ	81570
Pharosⁱ	Q9H078, Tbio
Protein Ontology More...PROⁱ	PR:Q9H078
RNActⁱ	Q9H078, protein
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000162129, Expressed in right testis and 182 other tissues
ExpressionAtlasⁱ	Q9H078, baseline and differential
Genevisibleⁱ	Q9H078, HS

Family and domain databases

Gene3Dⁱ	1.25.40.20, 2 hits
InterProⁱ	View protein in InterPro IPR003593, AAA+_ATPase IPR002110, Ankyrin_rpt IPR020683, Ankyrin_rpt-contain_dom IPR036770, Ankyrin_rpt-contain_sf IPR003959, ATPase_AAA_core IPR019489, Clp_ATPase_C IPR001270, ClpA/B IPR027417, P-loop_NTPase
Pfamⁱ	View protein in Pfam PF07724, AAA_2, 1 hit PF12796, Ank_2, 2 hits PF10431, ClpB_D2-small, 1 hit
PRINTSⁱ	PR00300, CLPPROTEASEA
SMARTⁱ	View protein in SMART SM00382, AAA, 1 hit SM00248, ANK, 3 hits SM01086, ClpB_D2-small, 1 hit
SUPFAMⁱ	SSF48403, SSF48403, 1 hit SSF52540, SSF52540, 1 hit
PROSITEⁱ	View protein in PROSITE PS50297, ANK_REP_REGION, 1 hit PS50088, ANK_REPEAT, 3 hits
ProtoNetⁱ	Search...
MobiDBⁱ	Search...

Entry informationⁱ

Entry nameⁱ	CLPB_HUMAN
Accessionⁱ	Q9H078Primary (citable) accession number: Q9H078 Secondary accession number(s): B4DXJ7 Q9H8Y0
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	November 8, 2002
	Last sequence update:	March 1, 2001
	Last modified:	August 12, 2020
	This is version 168 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

Reference proteome

Documents

Human chromosome 11
Human chromosome 11: entries, gene names and cross-references to MIM
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
SIMILARITY comments
Index of protein domains and families

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UniProtKB - Q9H078 (CLPB_HUMAN)

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Caseinolytic peptidase B protein homolog

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

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<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

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<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

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Keywords - PTMi

Proteomic databases

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<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Q9H078

Protein-protein interaction databases

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<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

<p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

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Keywords - Coding sequence diversityi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

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PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

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<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

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Functionⁱ

Catalytic activityⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ