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P10619

- PPGB_HUMAN

UniProt

P10619 - PPGB_HUMAN

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Protein

Lysosomal protective protein

Gene
CTSA, PPGB
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.1 Publication

Catalytic activityi

Release of a C-terminal amino acid with broad specificity.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei178 – 1781
Active sitei400 – 4001 By similarity
Active sitei457 – 4571

GO - Molecular functioni

  1. carboxypeptidase activity Source: ProtInc
  2. enzyme activator activity Source: ProtInc
  3. serine-type carboxypeptidase activity Source: InterPro

GO - Biological processi

  1. glycosphingolipid metabolic process Source: Reactome
  2. intracellular protein transport Source: ProtInc
  3. positive regulation of catalytic activity Source: GOC
  4. small molecule metabolic process Source: Reactome
  5. sphingolipid metabolic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Carboxypeptidase, Hydrolase, Protease

Enzyme and pathway databases

ReactomeiREACT_116105. Glycosphingolipid metabolism.
REACT_121399. MHC class II antigen presentation.
REACT_200874. Sialic acid metabolism.
SignaLinkiP10619.

Protein family/group databases

MEROPSiS10.002.

Names & Taxonomyi

Protein namesi
Recommended name:
Lysosomal protective protein (EC:3.4.16.5)
Alternative name(s):
Carboxypeptidase C
Carboxypeptidase L
Cathepsin A
Protective protein cathepsin A
Short name:
PPCA
Protective protein for beta-galactosidase
Cleaved into the following 2 chains:
Gene namesi
Name:CTSA
Synonyms:PPGB
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 20

Organism-specific databases

HGNCiHGNC:9251. CTSA.

Subcellular locationi

GO - Cellular componenti

  1. endoplasmic reticulum Source: ProtInc
  2. extracellular vesicular exosome Source: UniProt
  3. intracellular membrane-bounded organelle Source: HPA
  4. lysosomal lumen Source: Reactome
  5. lysosome Source: UniProtKB
  6. nucleus Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Lysosome

Pathology & Biotechi

Involvement in diseasei

Galactosialidosis (GSL) [MIM:256540]: A lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, secondary to a defect in cathepsin A. All patients have clinical manifestations typical of a lysosomal disorder, such as coarse facies, cherry red spots, vertebral changes, foam cells in the bone marrow, and vacuolated lymphocytes. Three phenotypic subtypes are recognized. The early infantile form is associated with fetal hydrops, edema, ascites, visceromegaly, skeletal dysplasia, and early death. The late infantile type is characterized by hepatosplenomegaly, growth retardation, cardiac involvement, and a normal or mildly affected mental state. The juvenile/adult form is characterized by myoclonus, ataxia, angiokeratoma, mental retardation, neurologic deterioration, absence of visceromegaly, and long survival.
Note: The disease is caused by mutations affecting the gene represented in this entry.4 Publications
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti49 – 491Q → R in GSL. 1 Publication
VAR_001385
Natural varianti51 – 511S → Y in GSL. 1 Publication
VAR_063018
Natural varianti65 – 651W → R in GSL. 1 Publication
Corresponds to variant rs28934603 [ dbSNP | Ensembl ].
VAR_001386
Natural varianti90 – 901S → L in GSL. 1 Publication
VAR_001387
Natural varianti132 – 1321V → M in GSL. 1 Publication
VAR_063019
Natural varianti236 – 2361L → P in GSL. 1 Publication
VAR_063020
Natural varianti249 – 2491Y → N in GSL; small amount of activity. 2 Publications
VAR_001388
Natural varianti395 – 3951Y → C in GSL; loss of activity. 1 Publication
VAR_001389
Natural varianti406 – 4061M → T in GSL. 1 Publication
VAR_063021
Natural varianti439 – 4391G → S in GSL. 1 Publication
VAR_063022
Natural varianti440 – 4401F → V in GSL. 2 Publications
VAR_001390
Natural varianti453 – 4531K → E in GSL. 1 Publication
VAR_063023

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi178 – 1781S → A: Inactivates the enzyme.
Mutagenesisi457 – 4571H → Q: Inactivates the enzyme.

Keywords - Diseasei

Disease mutation

Organism-specific databases

MIMi256540. phenotype.
Orphaneti351. Galactosialidosis.
PharmGKBiPA33572.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 28282 PublicationsAdd
BLAST
Chaini29 – 480452Lysosomal protective proteinPRO_0000004274Add
BLAST
Chaini29 – 326298Lysosomal protective protein 32 kDa chainPRO_0000004275Add
BLAST
Chaini327 – 480154Lysosomal protective protein 20 kDa chainPRO_0000004276Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi88 ↔ 362
Glycosylationi145 – 1451N-linked (GlcNAc...)2 Publications
Disulfide bondi240 ↔ 256
Disulfide bondi241 ↔ 246
Disulfide bondi281 ↔ 331
Glycosylationi333 – 3331N-linked (GlcNAc...)1 Publication

Keywords - PTMi

Disulfide bond, Glycoprotein, Zymogen

Proteomic databases

MaxQBiP10619.
PaxDbiP10619.
PRIDEiP10619.

2D gel databases

OGPiP10619.

PTM databases

PhosphoSiteiP10619.

Expressioni

Gene expression databases

ArrayExpressiP10619.
BgeeiP10619.
CleanExiHS_CTSA.
GenevestigatoriP10619.

Organism-specific databases

HPAiCAB024930.
HPA031068.

Interactioni

Subunit structurei

Heterodimer of a 32 kDa chain and a 20 kDa chain; disulfide-linked.

Protein-protein interaction databases

BioGridi111472. 20 interactions.
IntActiP10619. 8 interactions.
MINTiMINT-3007366.
STRINGi9606.ENSP00000361562.

Structurei

Secondary structure

1
480
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi31 – 333
Beta strandi41 – 433
Beta strandi49 – 557
Beta strandi60 – 678
Turni73 – 753
Beta strandi78 – 825
Turni85 – 873
Helixi91 – 966
Beta strandi99 – 1035
Beta strandi110 – 1123
Helixi117 – 1193
Beta strandi120 – 1267
Beta strandi136 – 1394
Helixi146 – 16318
Helixi165 – 1673
Beta strandi168 – 1703
Beta strandi172 – 1776
Helixi180 – 19213
Beta strandi199 – 2068
Helixi211 – 22414
Helixi230 – 24011
Helixi254 – 26815
Beta strandi269 – 2713
Beta strandi286 – 2927
Beta strandi295 – 2984
Helixi315 – 3173
Helixi318 – 3214
Beta strandi324 – 3274
Helixi335 – 3417
Helixi344 – 3496
Helixi364 – 3696
Beta strandi375 – 3773
Helixi378 – 3869
Beta strandi391 – 3977
Beta strandi401 – 4033
Helixi405 – 41410
Beta strandi424 – 4296
Helixi431 – 4333
Beta strandi435 – 44410
Beta strandi447 – 4526
Helixi459 – 4624
Helixi464 – 47512

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1IVYX-ray2.20A/B29-480[»]
3BP4X-ray1.85C2-10[»]
3BP7X-ray1.80C2-10[»]
3BXNX-ray1.86C2-10[»]
4AZ0X-ray2.17A29-326[»]
B327-480[»]
4AZ3X-ray2.04A29-326[»]
B327-480[»]
4CI9X-ray1.58A29-480[»]
4CIAX-ray1.98A29-480[»]
4CIBX-ray1.89A29-480[»]
4MWSX-ray2.80A/B29-480[»]
4MWTX-ray3.85A/B29-480[»]
ProteinModelPortaliP10619.
SMRiP10619. Positions 29-480.

Miscellaneous databases

EvolutionaryTraceiP10619.

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase S10 family.

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG2939.
HOVERGENiHBG053652.
InParanoidiP10619.
KOiK13289.
OrthoDBiEOG7KQ21H.
PhylomeDBiP10619.
TreeFamiTF323769.

Family and domain databases

Gene3Di3.40.50.1820. 1 hit.
InterProiIPR029058. AB_hydrolase.
IPR001563. Peptidase_S10.
IPR018202. Peptidase_S10_AS.
[Graphical view]
PANTHERiPTHR11802. PTHR11802. 1 hit.
PfamiPF00450. Peptidase_S10. 1 hit.
[Graphical view]
PRINTSiPR00724. CRBOXYPTASEC.
SUPFAMiSSF53474. SSF53474. 1 hit.
PROSITEiPS00560. CARBOXYPEPT_SER_HIS. 1 hit.
PS00131. CARBOXYPEPT_SER_SER. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. Align

Isoform 1 (identifier: P10619-1) [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

MIRAAPPPLF LLLLLLLLLV SWASRGEAAP DQDEIQRLPG LAKQPSFRQY    50
SGYLKGSGSK HLHYWFVESQ KDPENSPVVL WLNGGPGCSS LDGLLTEHGP 100
FLVQPDGVTL EYNPYSWNLI ANVLYLESPA GVGFSYSDDK FYATNDTEVA 150
QSNFEALQDF FRLFPEYKNN KLFLTGESYA GIYIPTLAVL VMQDPSMNLQ 200
GLAVGNGLSS YEQNDNSLVY FAYYHGLLGN RLWSSLQTHC CSQNKCNFYD 250
NKDLECVTNL QEVARIVGNS GLNIYNLYAP CAGGVPSHFR YEKDTVVVQD 300
LGNIFTRLPL KRMWHQALLR SGDKVRMDPP CTNTTAASTY LNNPYVRKAL 350
NIPEQLPQWD MCNFLVNLQY RRLYRSMNSQ YLKLLSSQKY QILLYNGDVD 400
MACNFMGDEW FVDSLNQKME VQRRPWLVKY GDSGEQIAGF VKEFSHIAFL 450
TIKGAGHMVP TDKPLAAFTM FSRFLNKQPY 480
Length:480
Mass (Da):54,466
Last modified:April 16, 2002 - v2
Checksum:i46B737DEE775C508
GO
Isoform 2 (identifier: P10619-2) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     102-118: Missing.

Note: Gene prediction based on EST data.

Show »
Length:463
Mass (Da):52,489
Checksum:i7342A76945E40684
GO

Sequence cautioni

The sequence CAI20248.1 differs from that shown. Reason: Erroneous initiation.

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti49 – 491Q → R in GSL. 1 Publication
VAR_001385
Natural varianti51 – 511S → Y in GSL. 1 Publication
VAR_063018
Natural varianti65 – 651W → R in GSL. 1 Publication
Corresponds to variant rs28934603 [ dbSNP | Ensembl ].
VAR_001386
Natural varianti90 – 901S → L in GSL. 1 Publication
VAR_001387
Natural varianti132 – 1321V → M in GSL. 1 Publication
VAR_063019
Natural varianti236 – 2361L → P in GSL. 1 Publication
VAR_063020
Natural varianti249 – 2491Y → N in GSL; small amount of activity. 2 Publications
VAR_001388
Natural varianti395 – 3951Y → C in GSL; loss of activity. 1 Publication
VAR_001389
Natural varianti406 – 4061M → T in GSL. 1 Publication
VAR_063021
Natural varianti439 – 4391G → S in GSL. 1 Publication
VAR_063022
Natural varianti440 – 4401F → V in GSL. 2 Publications
VAR_001390
Natural varianti453 – 4531K → E in GSL. 1 Publication
VAR_063023

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei102 – 11817Missing in isoform 2. VSP_054832Add
BLAST

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti19 – 191Missing in AAH00597. 1 Publication
Sequence conflicti56 – 561G → S in AAA36476. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
M22960 mRNA. Translation: AAA36476.1.
AK312898 mRNA. Translation: BAG35745.1.
AL008726 Genomic DNA. Translation: CAA15501.1.
AL008726 Genomic DNA. Translation: CAI20248.1. Different initiation.
BC000597 mRNA. Translation: AAH00597.1.
BC093009 mRNA. Translation: AAH93009.1.
CCDSiCCDS46609.1. [P10619-1]
PIRiA31589.
RefSeqiNP_000299.2. NM_000308.2.
NP_001121167.1. NM_001127695.1. [P10619-1]
NP_001161066.1. NM_001167594.1.
UniGeneiHs.609336.

Genome annotation databases

EnsembliENST00000191018; ENSP00000191018; ENSG00000064601.
ENST00000372459; ENSP00000361537; ENSG00000064601.
GeneIDi5476.
KEGGihsa:5476.
UCSCiuc002xqh.3. human. [P10619-1]

Polymorphism databases

DMDMi20178316.

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
M22960 mRNA. Translation: AAA36476.1 .
AK312898 mRNA. Translation: BAG35745.1 .
AL008726 Genomic DNA. Translation: CAA15501.1 .
AL008726 Genomic DNA. Translation: CAI20248.1 . Different initiation.
BC000597 mRNA. Translation: AAH00597.1 .
BC093009 mRNA. Translation: AAH93009.1 .
CCDSi CCDS46609.1. [P10619-1 ]
PIRi A31589.
RefSeqi NP_000299.2. NM_000308.2.
NP_001121167.1. NM_001127695.1. [P10619-1 ]
NP_001161066.1. NM_001167594.1.
UniGenei Hs.609336.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
1IVY X-ray 2.20 A/B 29-480 [» ]
3BP4 X-ray 1.85 C 2-10 [» ]
3BP7 X-ray 1.80 C 2-10 [» ]
3BXN X-ray 1.86 C 2-10 [» ]
4AZ0 X-ray 2.17 A 29-326 [» ]
B 327-480 [» ]
4AZ3 X-ray 2.04 A 29-326 [» ]
B 327-480 [» ]
4CI9 X-ray 1.58 A 29-480 [» ]
4CIA X-ray 1.98 A 29-480 [» ]
4CIB X-ray 1.89 A 29-480 [» ]
4MWS X-ray 2.80 A/B 29-480 [» ]
4MWT X-ray 3.85 A/B 29-480 [» ]
ProteinModelPortali P10619.
SMRi P10619. Positions 29-480.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 111472. 20 interactions.
IntActi P10619. 8 interactions.
MINTi MINT-3007366.
STRINGi 9606.ENSP00000361562.

Chemistry

BindingDBi P10619.
ChEMBLi CHEMBL6115.

Protein family/group databases

MEROPSi S10.002.

PTM databases

PhosphoSitei P10619.

Polymorphism databases

DMDMi 20178316.

2D gel databases

OGPi P10619.

Proteomic databases

MaxQBi P10619.
PaxDbi P10619.
PRIDEi P10619.

Protocols and materials databases

DNASUi 5476.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000191018 ; ENSP00000191018 ; ENSG00000064601 .
ENST00000372459 ; ENSP00000361537 ; ENSG00000064601 .
GeneIDi 5476.
KEGGi hsa:5476.
UCSCi uc002xqh.3. human. [P10619-1 ]

Organism-specific databases

CTDi 5476.
GeneCardsi GC20P044519.
HGNCi HGNC:9251. CTSA.
HPAi CAB024930.
HPA031068.
MIMi 256540. phenotype.
613111. gene.
neXtProti NX_P10619.
Orphaneti 351. Galactosialidosis.
PharmGKBi PA33572.
GenAtlasi Search...

Phylogenomic databases

eggNOGi COG2939.
HOVERGENi HBG053652.
InParanoidi P10619.
KOi K13289.
OrthoDBi EOG7KQ21H.
PhylomeDBi P10619.
TreeFami TF323769.

Enzyme and pathway databases

Reactomei REACT_116105. Glycosphingolipid metabolism.
REACT_121399. MHC class II antigen presentation.
REACT_200874. Sialic acid metabolism.
SignaLinki P10619.

Miscellaneous databases

ChiTaRSi CTSA. human.
EvolutionaryTracei P10619.
GeneWikii Cathepsin_A.
GenomeRNAii 5476.
NextBioi 21202.
PROi P10619.
SOURCEi Search...

Gene expression databases

ArrayExpressi P10619.
Bgeei P10619.
CleanExi HS_CTSA.
Genevestigatori P10619.

Family and domain databases

Gene3Di 3.40.50.1820. 1 hit.
InterProi IPR029058. AB_hydrolase.
IPR001563. Peptidase_S10.
IPR018202. Peptidase_S10_AS.
[Graphical view ]
PANTHERi PTHR11802. PTHR11802. 1 hit.
Pfami PF00450. Peptidase_S10. 1 hit.
[Graphical view ]
PRINTSi PR00724. CRBOXYPTASEC.
SUPFAMi SSF53474. SSF53474. 1 hit.
PROSITEi PS00560. CARBOXYPEPT_SER_HIS. 1 hit.
PS00131. CARBOXYPEPT_SER_SER. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Expression of cDNA encoding the human 'protective protein' associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases."
    Galjart N.J., Gillemans N., Harris A., van de Horst G.T.J., Verheijen F.W., Galjaard H., D'Azzo A.
    Cell 54:755-764(1988) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), PARTIAL PROTEIN SEQUENCE.
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Brain.
  3. "The DNA sequence and comparative analysis of human chromosome 20."
    Deloukas P., Matthews L.H., Ashurst J.L., Burton J., Gilbert J.G.R., Jones M., Stavrides G., Almeida J.P., Babbage A.K., Bagguley C.L., Bailey J., Barlow K.F., Bates K.N., Beard L.M., Beare D.M., Beasley O.P., Bird C.P., Blakey S.E.
    , Bridgeman A.M., Brown A.J., Buck D., Burrill W.D., Butler A.P., Carder C., Carter N.P., Chapman J.C., Clamp M., Clark G., Clark L.N., Clark S.Y., Clee C.M., Clegg S., Cobley V.E., Collier R.E., Connor R.E., Corby N.R., Coulson A., Coville G.J., Deadman R., Dhami P.D., Dunn M., Ellington A.G., Frankland J.A., Fraser A., French L., Garner P., Grafham D.V., Griffiths C., Griffiths M.N.D., Gwilliam R., Hall R.E., Hammond S., Harley J.L., Heath P.D., Ho S., Holden J.L., Howden P.J., Huckle E., Hunt A.R., Hunt S.E., Jekosch K., Johnson C.M., Johnson D., Kay M.P., Kimberley A.M., King A., Knights A., Laird G.K., Lawlor S., Lehvaeslaiho M.H., Leversha M.A., Lloyd C., Lloyd D.M., Lovell J.D., Marsh V.L., Martin S.L., McConnachie L.J., McLay K., McMurray A.A., Milne S.A., Mistry D., Moore M.J.F., Mullikin J.C., Nickerson T., Oliver K., Parker A., Patel R., Pearce T.A.V., Peck A.I., Phillimore B.J.C.T., Prathalingam S.R., Plumb R.W., Ramsay H., Rice C.M., Ross M.T., Scott C.E., Sehra H.K., Shownkeen R., Sims S., Skuce C.D., Smith M.L., Soderlund C., Steward C.A., Sulston J.E., Swann R.M., Sycamore N., Taylor R., Tee L., Thomas D.W., Thorpe A., Tracey A., Tromans A.C., Vaudin M., Wall M., Wallis J.M., Whitehead S.L., Whittaker P., Willey D.L., Williams L., Williams S.A., Wilming L., Wray P.W., Hubbard T., Durbin R.M., Bentley D.R., Beck S., Rogers J.
    Nature 414:865-871(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Placenta and Skin.
  5. "A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal 'protective protein'."
    Jackman H.L., Tan F., Tamei H., Beurling-Harbury C., Li X.-Y., Skidgel R.A., Erdoes E.G.
    J. Biol. Chem. 265:11265-11272(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 29-53 AND 327-351.
    Tissue: Platelet.
  6. "Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides."
    Gevaert K., Goethals M., Martens L., Van Damme J., Staes A., Thomas G.R., Vandekerckhove J.
    Nat. Biotechnol. 21:566-569(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 29-37.
    Tissue: Platelet.
  7. "Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function."
    Galjart N.J., Morreau H., Willemsen R., Gillemans N., Bonten E.J., D'Azzo A.
    J. Biol. Chem. 266:14754-14762(1991) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, MUTAGENESIS.
  8. "Elucidation of N-glycosylation sites on human platelet proteins: a glycoproteomic approach."
    Lewandrowski U., Moebius J., Walter U., Sickmann A.
    Mol. Cell. Proteomics 5:226-233(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-145.
    Tissue: Platelet.
  9. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
    Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.
    J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-145 AND ASN-333.
    Tissue: Liver.
  10. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  11. "Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism."
    Rudenko G., Bonten E., D'Azzo A., Hol W.G.J.
    Structure 3:1249-1259(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: X-RAY CRYSTALLOGRAPHY (2.2 ANGSTROMS).
  12. "A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable."
    Zhou X.Y., Galjart N.J., Willemsen R., Gillemans N., Galjaard H., D'Azzo A.
    EMBO J. 10:4041-4048(1991) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANT GSL VAL-440.
  13. Cited for: VARIANTS GSL ARG-49; ARG-65; LEU-90; ASN-249 AND CYS-395.
  14. "Molecular and biochemical analysis of protective protein/cathepsin A mutations: correlation with clinical severity in galactosialidosis."
    Zhou X.Y., van der Spoel A., Rottier R., Hale G., Willemsen R., Berry G.T., Strisciuglio P., Morrone A., Zammarchi E., Andria G., d'Azzo A.
    Hum. Mol. Genet. 5:1977-1987(1996) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANTS GSL TYR-51; MET-132; PRO-236; ASN-249; THR-406; SER-439 AND VAL-440.
  15. "Structural and functional study of K453E mutant protective protein/cathepsin A causing the late infantile form of galactosialidosis."
    Takiguchi K., Itoh K., Shimmoto M., Ozand P.T., Doi H., Sakuraba H.
    J. Hum. Genet. 45:200-206(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANT GSL GLU-453.

Entry informationi

Entry nameiPPGB_HUMAN
AccessioniPrimary (citable) accession number: P10619
Secondary accession number(s): B2R798
, Q561W6, Q5JZH1, Q96KJ2, Q9BR08, Q9BW68
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: April 16, 2002
Last modified: September 3, 2014
This is version 179 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 20
    Human chromosome 20: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Peptidase families
    Classification of peptidase families and list of entries
  7. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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