Cathepsin A

PDB rendering based on 1ivy.

Available structures
PDB	1IVY, 3BP4, 3BP7, 3BXN

Identifiers

Symbols

CTSA; GLB2; GSL; NGBE; PPCA; PPGB

External IDs

OMIM: 613111 MGI: 97748 HomoloGene: 80163 GeneCards: CTSA Gene

EC number

3.4.16.5

Gene Ontology
Molecular function	• carboxypeptidase activity • serine-type carboxypeptidase activity • protein binding • enzyme activator activity • peptidase activity
Cellular component	• nucleus • nucleolus • mitochondrion • lysosome • endoplasmic reticulum • intracellular membrane-bounded organelle
Biological process	• proteolysis • intracellular protein transport • positive regulation of catalytic activity
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 20:
44.52 – 44.53 Mb

Chr 2:
164.66 – 164.67 Mb

PubMed search

[1]

[2]

Cathepsin A is an enzyme which is classified both as a cathepsin and a carboxypeptidase. In humans, it is encoded by the CTSA gene.^[1]

1 Function
2 Clinical significance
3 Interactions
4 References
5 Further reading
6 External links

Function

This gene encodes a glycoprotein which associates with lysosomal enzymes beta-galactosidase and neuraminidase to form a complex of high molecular weight multimers. The formation of this complex provides a protective role for stability and activity. It is protective for β-galactosidase and neuraminidase.^[2]

Clinical significance

Deficiencies in this gene are linked to multiple forms of galactosialidosis.^[1]

Interactions

Cathepsin A has been shown to interact with NEU1.^[3]

References

^ ^a ^b "Entrez Gene: CTSA cathepsin A". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=5476.
^ Mitchell, Richard Sheppard; Kumar, Vinay; Robbins, Stanley L.; Abbas, Abul K.; Fausto, Nelson (2007). "Table 7-6". Robbins basic pathology (8th ed.). Saunders/Elsevier. ISBN 1-4160-2973-7.
^ van der Spoel, A; Bonten E, d'Azzo A (Mar. 1998). "Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A". EMBO J. (ENGLAND) 17 (6): 1588–97. doi:10.1093/emboj/17.6.1588. ISSN 0261-4189. PMC 1170506. PMID 9501080. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1170506.

Morreau H, Galjart NJ, Willemsen R, et al. (1992). "Human lysosomal protective protein. Glycosylation, intracellular transport, and association with beta-galactosidase in the endoplasmic reticulum.". J. Biol. Chem. 267 (25): 17949–56. PMID 1387645.
Halal F, Chitayat D, Parikh H, et al. (1992). "Ring chromosome 20 and possible assignment of the structural gene encoding human carboxypeptidase-L to the distal segment of the long arm of chromosome 20.". Am. J. Med. Genet. 43 (3): 576–9. doi:10.1002/ajmg.1320430314. PMID 1605251.
Jackman HL, Tan FL, Tamei H, et al. (1990). "A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal "protective protein".". J. Biol. Chem. 265 (19): 11265–72. PMID 1694176.
Zhou XY, Galjart NJ, Willemsen R, et al. (1992). "A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable.". EMBO J. 10 (13): 4041–8. PMC 453152. PMID 1756715. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=453152.
Galjart NJ, Morreau H, Willemsen R, et al. (1991). "Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function.". J. Biol. Chem. 266 (22): 14754–62. PMID 1907282.
Yoshida K, Oshima A, Shimmoto M, et al. (1991). "Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases.". Am. J. Hum. Genet. 49 (2): 435–42. PMC 1683306. PMID 1907800. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1683306.
Wiegant J, Galjart NJ, Raap AK, d'Azzo A (1991). "The gene encoding human protective protein (PPGB) is on chromosome 20.". Genomics 10 (2): 345–9. doi:10.1016/0888-7543(91)90318-9. PMID 2071143.
Strisciuglio P, Sly WS, Dodson WE, et al. (1991). "Combined deficiency of beta-galactosidase and neuraminidase: natural history of the disease in the first 18 years of an American patient with late infantile onset form.". Am. J. Med. Genet. 37 (4): 573–7. doi:10.1002/ajmg.1320370431. PMID 2148053.
Kase R, Itoh K, Takiyama N, et al. (1990). "Galactosialidosis: simultaneous deficiency of esterase, carboxy-terminal deamidase and acid carboxypeptidase activities.". Biochem. Biophys. Res. Commun. 172 (3): 1175–9. doi:10.1016/0006-291X(90)91572-A. PMID 2244901.
Willemsen R, Hoogeveen AT, Sips HJ, et al. (1986). "Immunoelectron microscopical localization of lysosomal beta-galactosidase and its precursor forms in normal and mutant human fibroblasts.". Eur. J. Cell Biol. 40 (1): 9–15. PMID 3084261.
Verheijen FW, Palmeri S, Galjaard H (1987). "Purification and partial characterization of lysosomal neuraminidase from human placenta.". Eur. J. Biochem. 162 (1): 63–7. doi:10.1111/j.1432-1033.1987.tb10542.x. PMID 3102233.
Nanba E, Tsuji A, Omura K, Suzuki Y (1987). "Galactosialidosis: a direct evidence that a 46-kilodalton protein restores deficient enzyme activities in fibroblasts.". Biochem. Biophys. Res. Commun. 144 (1): 138–42. doi:10.1016/S0006-291X(87)80486-2. PMID 3107551.
Galjart NJ, Gillemans N, Harris A, et al. (1988). "Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases.". Cell 54 (6): 755–64. doi:10.1016/S0092-8674(88)90999-3. PMID 3136930.
Chitayat D, Applegarth DA, Lewis J, et al. (1989). "Juvenile galactosialidosis in a white male: a new variant.". Am. J. Med. Genet. 31 (4): 887–901. doi:10.1002/ajmg.1320310423. PMID 3149149.
Verheijen FW, Palmeri S, Hoogeveen AT, Galjaard H (1985). "Human placental neuraminidase. Activation, stabilization and association with beta-galactosidase and its protective protein.". Eur. J. Biochem. 149 (2): 315–21. doi:10.1111/j.1432-1033.1985.tb08928.x. PMID 3922758.
van der Horst GT, Kleijer WJ, Hoogeveen AT, et al. (1984). "Morquio B syndrome: a primary defect in beta-galactosidase.". Am. J. Med. Genet. 16 (2): 261–75. doi:10.1002/ajmg.1320160215. PMID 6418007.
Maire I, Nivelon-Chevallier AR (1982). "Combined deficiency of beta-galactosidase and neuraminidase: three affected siblings in a French family.". J. Inherit. Metab. Dis. 4 (4): 221–3. doi:10.1007/BF02263656. PMID 6796775.
Pshezhetsky AV, Potier M (1994). "Direct affinity purification and supramolecular organization of human lysosomal cathepsin A.". Arch. Biochem. Biophys. 313 (1): 64–70. doi:10.1006/abbi.1994.1359. PMID 8053688.
Ishii N, Oshima A, Sakuraba H, et al. (1994). "Normal serum beta-galactosidase in juvenile GM1 gangliosidosis.". Pediatr. Neurol. 10 (4): 317–9. doi:10.1016/0887-8994(94)90129-5. PMID 8068159.
Chakraborty S, Rafi MA, Wenger DA (1994). "Mutations in the lysosomal beta-galactosidase gene that cause the adult form of GM1 gangliosidosis.". Am. J. Hum. Genet. 54 (6): 1004–13. PMC 1918177. PMID 8198123. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1918177.

External links

MeSH Cathepsin+A

PDB gallery

1ivy: PHYSIOLOGICAL DIMER HPP PRECURSOR

Endopeptidases: serine proteases/serine endopeptidases (EC 3.4.21)

Digestive enzymes	Enteropeptidase · Trypsin · Chymotrypsin · Elastase (Neutrophil, Pancreatic)

Coagulation	factors: Thrombin · Factor VIIa · Factor IXa · Factor Xa · Factor XIa · Factor XIIa · Kallikrein (PSA, KLK1, KLK2, KLK3, KLK4, KLK5, KLK6, KLK7, KLK8, KLK9, KLK10, KLK11, KLK12, KLK13, KLK14, KLK15) fibrinolysis: Plasmin · Plasminogen activator (Tissue plasminogen activator · Urinary plasminogen activator)

Complement system	Factor B · Factor D · Factor I · MASP (MASP1, MASP2) · C3-convertase

Other immune system	Chymase · Granzyme · Tryptase · Proteinase 3/Myeloblastin

Venombin	Ancrod · Batroxobin

Other	Acrosin · Prolyl endopeptidase · Pronase · Proprotein convertases (1, 2) · Reelin · Subtilisin/Furin · Streptokinase · S1P · Cathepsin (A, G)

B enzm: 1.1/2/3/4/5/6/7/8/10/11/13/14/15-18, 2.1/2/3/4/5/6/7/8, 2.7.10, 2.7.11-12, 3.1/2/3/4/5/6/7, 3.1.3.48, 3.4.21/22/23/24, 4.1/2/3/4/5/6, 5.1/2/3/4/99, 6.1-3/4/5-6