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Protein

Cytosolic carboxypeptidase 1

Gene

Agtpbp1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Metallocarboxypeptidase that mediates deglutamylation of target proteins. Catalyzes the deglutamylation of polyglutamate side chains generated by post-translational polyglutamylation in proteins such as tubulins. Also removes gene-encoded polyglutamates from the carboxy-terminus of target proteins such as MYLK. Acts as a long-chain deglutamylase and specifically shortens long polyglutamate chains, while it is not able to remove the branching point glutamate, a process catalyzed by AGBL5/CCP5. Deglutamylation plays a key role in cerebellar Purkinje cell differentiation, accumulation of tubulin polyglutamylation causing neurodegeneration.2 Publications

Cofactori

Zn2+By similarityNote: Binds 1 zinc ion per subunit.By similarity

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi912 – 9121ZincCurated
Metal bindingi915 – 9151ZincCurated
Active sitei962 – 9621NucleophileBy similarity
Metal bindingi1009 – 10091ZincBy similarity

GO - Molecular functioni

  • metallocarboxypeptidase activity Source: UniProtKB
  • peptidase activity Source: UniProtKB
  • tubulin binding Source: UniProtKB
  • zinc ion binding Source: InterPro

GO - Biological processi

  • adult walking behavior Source: MGI
  • cerebellar Purkinje cell differentiation Source: UniProtKB
  • cerebellar Purkinje cell layer development Source: MGI
  • cerebellum development Source: MGI
  • C-terminal protein deglutamylation Source: UniProtKB
  • eye photoreceptor cell differentiation Source: UniProtKB
  • mitochondrion organization Source: UniProtKB
  • neuromuscular process Source: UniProtKB
  • neurotransmitter metabolic process Source: MGI
  • olfactory bulb development Source: UniProtKB
  • protein side chain deglutamylation Source: UniProtKB
  • retina development in camera-type eye Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Carboxypeptidase, Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Metal-binding, Zinc

Names & Taxonomyi

Protein namesi
Recommended name:
Cytosolic carboxypeptidase 1 (EC:3.4.17.-)
Alternative name(s):
ATP/GTP-binding protein 1
Nervous system nuclear protein induced by axotomy protein 1
Gene namesi
Name:Agtpbp1
Synonyms:Ccp1, Nna1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 13

Organism-specific databases

MGIiMGI:2159437. Agtpbp1.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: MGI
  • cytosol Source: UniProtKB
  • mitochondrion Source: UniProtKB
  • nucleus Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion, Nucleus

Pathology & Biotechi

Involvement in diseasei

Defects in Agtpbp1 are the cause of Purkinje cell degeneration (pcd). Pcd is a spontaneous mutation that causes adult-onset degeneration of cerebellar Purkinje neurons, retinal photoreceptors, olfactory bulb mitral neurons, and selected thalamic neurons, and has defective spermatogenesis. Defects in mitochondrial metabolic functions are also observed. The molecular causes of neurodegeneration are still unclear, but they are probably due to an accumulation of glutamylation, either tubulin hyperglutamylation or another hyperglutamylated target proteins. An increase of intranuclear localization of lysyl oxidase (Lox) propeptide, which interferes with NF-kappa-B Rela signaling and microtubule-associated protein regulation of microtubule stability is also observed, possibly leading to underdevelopment of Purkinje cell dendrites.

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi912 – 9121H → A: Abolishes ability to rescue Purkinje cell degeneration in pcd mice when expressed in a transgene. 3 Publications
Mutagenesisi912 – 9121H → S: Abolishes deglutamylase activity; when associated with Q-915. 3 Publications
Mutagenesisi915 – 9151E → A: Abolishes ability to rescue Purkinje cell degeneration in pcd mice when expressed in a transgene. 3 Publications
Mutagenesisi915 – 9151E → Q: Abolishes deglutamylase activity; when associated with S-912. 3 Publications
Mutagenesisi971 – 9722NR → AA: Abolishes ability to rescue Purkinje cell degeneration in pcd mice when expressed in a transgene. 1 Publication

Keywords - Diseasei

Disease mutation, Neurodegeneration

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 12181218Cytosolic carboxypeptidase 1PRO_0000308691Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei1160 – 11601PhosphoserineBy similarity

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ641K1.
MaxQBiQ641K1.
PaxDbiQ641K1.
PRIDEiQ641K1.

PTM databases

iPTMnetiQ641K1.
PhosphoSiteiQ641K1.

Expressioni

Tissue specificityi

Widely expressed. Highly expressed in the cerebellum and cortex of adult mouse brain. Expressed at similar levels in both the cerebellum and the cortex throughout all developmental stages. Also expressed in sciatic nerve transection, spinal motor neurons undergoing axon regeneration, testis, heart and in developing brain. Expression in cranial motor nuclei is the same as that observed in uninjured primary motor neurons. Expression is prevalent in sensory neurons and hippocampal CA3 neurons in addition to regenerating motor neurons.3 Publications

Developmental stagei

Highly expressed in differentiating neurons. From E16.5, expression is widespread in brain, spinal cord, and peripheral nervous tissue. Within the developing CNS, expression is restricted to regions of brain and spinal cord containing differentiating neurons.1 Publication

Inductioni

By axonal regeneration.2 Publications

Gene expression databases

BgeeiENSMUSG00000021557.
CleanExiMM_AGTPBP1.
ExpressionAtlasiQ641K1. baseline and differential.
GenevisibleiQ641K1. MM.

Interactioni

Subunit structurei

Interacts with MYLK.1 Publication

GO - Molecular functioni

  • tubulin binding Source: UniProtKB

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000022040.

Structurei

3D structure databases

ProteinModelPortaliQ641K1.
SMRiQ641K1. Positions 701-1024.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase M14 family.Curated

Phylogenomic databases

eggNOGiKOG3641. Eukaryota.
COG2866. LUCA.
GeneTreeiENSGT00550000074405.
HOVERGENiHBG107587.
InParanoidiQ641K1.
OMAiHIAPAFC.
OrthoDBiEOG091G01L5.
PhylomeDBiQ641K1.
TreeFamiTF313794.

Family and domain databases

Gene3Di1.25.10.10. 1 hit.
InterProiIPR011989. ARM-like.
IPR016024. ARM-type_fold.
IPR000834. Peptidase_M14.
[Graphical view]
PfamiPF00246. Peptidase_M14. 1 hit.
[Graphical view]
SUPFAMiSSF48371. SSF48371. 1 hit.

Sequences (5)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q641K1-1) [UniParc]FASTAAdd to basket

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

« Hide

        10         20         30         40         50
MSKLKVVGEK SLTNSSRVVG LLAQLEKINT DSTESDTARY VTSKILHLAQ
60 70 80 90 100
SQEKTRREMT TKGSTGMEVL LSTLENTKDL QTVLNILSIL IELVSSGGGR
110 120 130 140 150
RASFLVAKGG SQILLQLLMN ASKDSPPHEE VMVQTHSILA KIGPKDKKFG
160 170 180 190 200
VKARVNGALT VTLNLVKQHF QNYRLVLPCL QLLRVYSTNS VNSVSLGKNG
210 220 230 240 250
VVELMFKIIG PFSKKNSGLM KVALDTLAAL LKSKTNARRA VDRGYVQVLL
260 270 280 290 300
TIYVDWHRHD NRHRNMLIRK GILQSLKSVT NIKLGRKAFI DANGMKILYN
310 320 330 340 350
TSQECLAVRT LDPLVNTSSL IMRKCFPKNR LPLPTIKSSF HFQLPIIPVT
360 370 380 390 400
GPVAQLYSLP PEVDDVVDES DDNDDIDLEV ENELENEDDL DQSFKNDDIE
410 420 430 440 450
TDINKLRPQQ VPGRTIEELK MYEHLFPELV DDFQDYELIS KEPKPFVFEG
460 470 480 490 500
KARGPIVVPT AGEEVPGNSG SVKKGVVMKE RASPKGEEAK EDPKGHDRTL
510 520 530 540 550
PQQLGGQSRV APSAHSFNND LVKALDRITL QNVPSQVASG LNAGMRKDFG
560 570 580 590 600
LPLTVLSCTK ACPHVAKCGS TLFEGRTVHL GKLCCTGVET EDDEDTESHS
610 620 630 640 650
STEQAPSVEA SDGPTLHDPD LYIEIVKNTK SVPEYSEVAY PDYFGHIPPP
660 670 680 690 700
FKEPILERPY GVQRTKIAQD IERLIHQNDI IDRVVYDLDN PTYTTPEEGD
710 720 730 740 750
TLKFNSKFES GNLRKVIQIR KSEYDLILNS DINSNHYHQW FYFEVSGMRP
760 770 780 790 800
GVAYRFNIIN CEKSNSQFNY GMQPLMYSVQ EALNARPWWI RMGTDICYYK
810 820 830 840 850
NHFSRSSVAA GGQKGKSYYT ITFTVNFPHK DDVCYFAYHY PYTYSTLQMH
860 870 880 890 900
LQKLESAHNP QQIYFRKDVL CETLSGNICP LVTITAMPES NYYEHICQFR
910 920 930 940 950
TRPYIFLSAR VHPGETNASW VMKGTLEYLM SNSPTAQSLR ESYIFKIVPM
960 970 980 990 1000
LNPDGVINGN HRCSLSGEDL NRQWQSPNPE LHPTIYHAKG LLQYLAAVKR
1010 1020 1030 1040 1050
LPLVYCDYHG HSRKKNVFMY GCSIKETVWH THDNSASCDI VEDMGYRTLP
1060 1070 1080 1090 1100
KILSHIAPAF CMSSCSFVVE KSKESTARVV VWREIGVQRS YTMESTLCGC
1110 1120 1130 1140 1150
DQGRYKGLQI GTRELEEMGA KFCVGLLRLK RLTSSLEYNL PSNLLDFEND
1160 1170 1180 1190 1200
LIESSCKVTS PTTYVLDEDE PRFLEEVDYS AESNDELDVE LAENTGDYEP
1210
SAQEEALSDS EVSRTHLI
Length:1,218
Mass (Da):137,197
Last modified:October 23, 2007 - v2
Checksum:i89917C6897AD9DD5
GO
Isoform 2 (identifier: Q641K1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1160-1174: SPTTYVLDEDEPRFL → RTRGSSELQLFPAVL
     1175-1218: Missing.

Note: No experimental confirmation available.
Show »
Length:1,174
Mass (Da):132,220
Checksum:iD1C7D2CFAFB5D0EA
GO
Isoform 3 (identifier: Q641K1-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     771-795: GMQPLMYSVQEALNARPWWIRMGTD → EITSHEAQLPQADRRASPTTPSPSP
     796-1218: Missing.

Note: No experimental confirmation available. Apparent retained intron. May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Show »
Length:795
Mass (Da):88,657
Checksum:iDE3C127579DC21F3
GO
Isoform 4 (identifier: Q641K1-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     771-789: GMQPLMYSVQEALNARPWW → DGEETCYKMIVVSTICCKD
     790-1218: Missing.

Note: No experimental confirmation available.
Show »
Length:789
Mass (Da):88,120
Checksum:iF765D2CD582ABE31
GO
Isoform 5 (identifier: Q641K1-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     689-1218: Missing.

Note: No experimental confirmation available.
Show »
Length:688
Mass (Da):76,297
Checksum:iB1779CDF33BDDE52
GO

Sequence cautioni

The sequence AAG37102 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti237 – 2371A → V in AAG37102 (PubMed:11083920).Curated
Sequence conflicti274 – 2741Q → R in AAG37102 (PubMed:11083920).Curated
Sequence conflicti380 – 3801V → A in BC060633 (PubMed:15489334).Curated
Sequence conflicti542 – 5487NAGMRKD → ERRNEEG in AAG37102 (PubMed:11083920).Curated
Sequence conflicti644 – 6441F → S in AAG37102 (PubMed:11083920).Curated
Sequence conflicti969 – 9691D → G in AAG37102 (PubMed:11083920).Curated
Sequence conflicti1043 – 10431D → G in AAG37102 (PubMed:11083920).Curated
Sequence conflicti1121 – 11211K → Q in AAG37102 (PubMed:11083920).Curated
Sequence conflicti1126 – 11261L → I in BAC25412 (PubMed:16141072).Curated
Sequence conflicti1167 – 11671D → G in BAB24963 (PubMed:16141072).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti832 – 8321D → DD in pcd; pcd(5J) mutant. 1 Publication

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei689 – 1218530Missing in isoform 5. 1 PublicationVSP_038804Add
BLAST
Alternative sequencei771 – 79525GMQPL…RMGTD → EITSHEAQLPQADRRASPTT PSPSP in isoform 3. 1 PublicationVSP_029045Add
BLAST
Alternative sequencei771 – 78919GMQPL…ARPWW → DGEETCYKMIVVSTICCKD in isoform 4. 1 PublicationVSP_038803Add
BLAST
Alternative sequencei790 – 1218429Missing in isoform 4. 1 PublicationVSP_038805Add
BLAST
Alternative sequencei796 – 1218423Missing in isoform 3. 1 PublicationVSP_029046Add
BLAST
Alternative sequencei1160 – 117415SPTTY…EPRFL → RTRGSSELQLFPAVL in isoform 2. 1 PublicationVSP_029047Add
BLAST
Alternative sequencei1175 – 121844Missing in isoform 2. 1 PublicationVSP_029048Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF219141 mRNA. Translation: AAG37102.1. Different initiation.
AK007328 mRNA. Translation: BAB24963.2.
AK013688 mRNA. Translation: BAC25412.1.
AK132695 mRNA. Translation: BAE21306.1.
AK170046 mRNA. Translation: BAE41530.1.
BC082335 mRNA. Translation: AAH82335.1.
BC060633 mRNA. No translation available.
CCDSiCCDS36686.1. [Q641K1-1]
CCDS36687.1. [Q641K1-4]
CCDS70470.1. [Q641K1-2]
RefSeqiNP_001041473.1. NM_001048008.2. [Q641K1-4]
NP_001271147.1. NM_001284218.1. [Q641K1-4]
NP_001271148.1. NM_001284219.1.
NP_001271150.1. NM_001284221.1. [Q641K1-2]
NP_075817.2. NM_023328.3. [Q641K1-1]
XP_006517398.1. XM_006517335.1. [Q641K1-1]
XP_006517399.1. XM_006517336.2. [Q641K1-1]
XP_006517400.1. XM_006517337.2. [Q641K1-1]
XP_006517401.1. XM_006517338.2. [Q641K1-1]
XP_011242857.1. XM_011244555.1. [Q641K1-2]
UniGeneiMm.153008.

Genome annotation databases

EnsembliENSMUST00000022040; ENSMUSP00000022040; ENSMUSG00000021557. [Q641K1-1]
ENSMUST00000109830; ENSMUSP00000105456; ENSMUSG00000021557. [Q641K1-4]
ENSMUST00000164215; ENSMUSP00000130939; ENSMUSG00000021557. [Q641K1-2]
ENSMUST00000170555; ENSMUSP00000128589; ENSMUSG00000021557. [Q641K1-3]
ENSMUST00000171606; ENSMUSP00000132697; ENSMUSG00000021557. [Q641K1-4]
GeneIDi67269.
KEGGimmu:67269.
UCSCiuc007qum.1. mouse. [Q641K1-1]
uc007quq.2. mouse. [Q641K1-4]
uc033gmf.1. mouse. [Q641K1-2]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF219141 mRNA. Translation: AAG37102.1. Different initiation.
AK007328 mRNA. Translation: BAB24963.2.
AK013688 mRNA. Translation: BAC25412.1.
AK132695 mRNA. Translation: BAE21306.1.
AK170046 mRNA. Translation: BAE41530.1.
BC082335 mRNA. Translation: AAH82335.1.
BC060633 mRNA. No translation available.
CCDSiCCDS36686.1. [Q641K1-1]
CCDS36687.1. [Q641K1-4]
CCDS70470.1. [Q641K1-2]
RefSeqiNP_001041473.1. NM_001048008.2. [Q641K1-4]
NP_001271147.1. NM_001284218.1. [Q641K1-4]
NP_001271148.1. NM_001284219.1.
NP_001271150.1. NM_001284221.1. [Q641K1-2]
NP_075817.2. NM_023328.3. [Q641K1-1]
XP_006517398.1. XM_006517335.1. [Q641K1-1]
XP_006517399.1. XM_006517336.2. [Q641K1-1]
XP_006517400.1. XM_006517337.2. [Q641K1-1]
XP_006517401.1. XM_006517338.2. [Q641K1-1]
XP_011242857.1. XM_011244555.1. [Q641K1-2]
UniGeneiMm.153008.

3D structure databases

ProteinModelPortaliQ641K1.
SMRiQ641K1. Positions 701-1024.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000022040.

PTM databases

iPTMnetiQ641K1.
PhosphoSiteiQ641K1.

Proteomic databases

EPDiQ641K1.
MaxQBiQ641K1.
PaxDbiQ641K1.
PRIDEiQ641K1.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000022040; ENSMUSP00000022040; ENSMUSG00000021557. [Q641K1-1]
ENSMUST00000109830; ENSMUSP00000105456; ENSMUSG00000021557. [Q641K1-4]
ENSMUST00000164215; ENSMUSP00000130939; ENSMUSG00000021557. [Q641K1-2]
ENSMUST00000170555; ENSMUSP00000128589; ENSMUSG00000021557. [Q641K1-3]
ENSMUST00000171606; ENSMUSP00000132697; ENSMUSG00000021557. [Q641K1-4]
GeneIDi67269.
KEGGimmu:67269.
UCSCiuc007qum.1. mouse. [Q641K1-1]
uc007quq.2. mouse. [Q641K1-4]
uc033gmf.1. mouse. [Q641K1-2]

Organism-specific databases

CTDi23287.
MGIiMGI:2159437. Agtpbp1.

Phylogenomic databases

eggNOGiKOG3641. Eukaryota.
COG2866. LUCA.
GeneTreeiENSGT00550000074405.
HOVERGENiHBG107587.
InParanoidiQ641K1.
OMAiHIAPAFC.
OrthoDBiEOG091G01L5.
PhylomeDBiQ641K1.
TreeFamiTF313794.

Miscellaneous databases

PROiQ641K1.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000021557.
CleanExiMM_AGTPBP1.
ExpressionAtlasiQ641K1. baseline and differential.
GenevisibleiQ641K1. MM.

Family and domain databases

Gene3Di1.25.10.10. 1 hit.
InterProiIPR011989. ARM-like.
IPR016024. ARM-type_fold.
IPR000834. Peptidase_M14.
[Graphical view]
PfamiPF00246. Peptidase_M14. 1 hit.
[Graphical view]
SUPFAMiSSF48371. SSF48371. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiCBPC1_MOUSE
AccessioniPrimary (citable) accession number: Q641K1
Secondary accession number(s): Q3TDS0
, Q3V147, Q6P9R9, Q8C1K8, Q9D962, Q9EQI4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 23, 2007
Last sequence update: October 23, 2007
Last modified: September 7, 2016
This is version 101 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.