human-CTSA

 
Homo sapiens (Human) protective protein associated with lysosomal beta-galactosidase ppt2 protein CTSA Cathepsin A, PPGB

Comment
Cathepsin A (CTSA , CATHA protective protein PPCA, beta-galactosidase protective protein PPGB, Carboxypeptidase L, Carboxypeptidase C, Protective protein cathepsin A) is a ubiquitously expressed multifunctional enzyme, with deamidase, esterase, and carboxypeptidase activities and a preference for substrates with hydrophobic amino acid residues at the P1-prime position. Association with CTSA, as part of the lysosomal multienzyme complex, is essential for stabilization of lysosomal beta-galactosidase (GLB1; 611458), as well as for activation of the lysosomal neuraminidase (NEU1; 608272) (summary by Seyrantepe et al., 2008). The protective protein is a glycoprotein that associates with lysosomal beta-galactosidase and neuraminidase and is deficient in the autosomal recessive disorder Galactosialidosis. BC000597 Trembl Q9BW68 clone MGC:1994 Similar to protective protein for beta-galactosidase aussi EC 3.4.16.5, Cathepsin A, Carboxypeptidase C, AL008726 Trembl Q96KJ2 HS337O18_11 gene isoform 1, AL008726 Trembl Q9BR08 HS337O18_10 gene isoform 2


Relationship
Block X
Homo sapiens position in NCBI Life Tree :
N link to NCBI taxonomic web page and E link to ESTHER gene locus found in this strain.
> cellular organisms: N E > Eukaryota: N E > Opisthokonta: N E > Metazoa: N E > Eumetazoa: N E > Bilateria: N E > Deuterostomia: N E > Chordata: N E > Craniata: N E > Vertebrata: N E > Gnathostomata: N E > Teleostomi: N E > Euteleostomi: N E > Sarcopterygii: N E > Dipnotetrapodomorpha: N E > Tetrapoda: N E > Amniota: N E > Mammalia: N E > Theria: N E > Eutheria: N E > Boreoeutheria: N E > Euarchontoglires: N E > Primates: N E > Haplorrhini: N E > Simiiformes: N E > Catarrhini: N E > Hominoidea: N E > Hominidae: N E > Homininae: N E > Homo: N E > Homo sapiens: N E

Molecular evidence
Database
25 mutations: Table (ordered Natural and SD mutagenesis) (e.g. : F440V_human-CTSA, IVS7ex7del_human-CTSA, Q49R_human-CTSA ... more)
9 structures (e.g. : 1IVY, 4CIA, 4AZ0... more)
No kinetic
Disease: Galactosialidosis -
Substrate: Tenofovir-alafenamide , Remdesivir ,
Inhibitor: 2-(cyclohexylmethyl)propanedioic-acid , Rocaglate-Derived-beta-Lactone-5 , CHEMBL3145341 , CHEMBL2171392 , VX-Biotin , Cyclosarin , DFP , Methylparaoxon , Sarin , Soman ,
>3 Genbank links 5 more: AB209705, BC093009, AL008726
>3 UniProt links 1 more: P10619, Q5JZH0, Q59EV6
2 Ncbi-nid : 190282, 12653638
1 Ncbi-pid : 12653639
>3 Structure links 6 more: 1IVY, 4CIA, 4AZ0
>3 UniProtTrembl links 1 more: P10619, Q5JZH0, Q59EV6
>3 Interpro links 1 more: P10619, Q5JZH0, Q59EV6
>3 Prodom links 1 more: P10619, Q5JZH0, Q59EV6
>3 Pfam links 1 more: P10619, Q5JZH0, Q59EV6
>3 PIRSF links 1 more: P10619, Q5JZH0, Q59EV6
>3 SUPERFAM links 1 more: P10619, Q5JZH0, Q59EV6
>3 QuickSwissBlast links 1 more: P10619, Q5JZH0, Q59EV6
1 EntrezGene : 5476
1 SNP : 5476
1 UniGene : 609336
1 HUGO HGNC : 9251
1 IUPHAR : 1581
2 OMIM : 256540, 613111
1 Ensembl : ENSG00000064601
 
Sequence
Graphical view for this peptide sequence: human-CTSA
Colored MSA for Carboxypeptidase_S10 (raw)
MIRAAPPPLFLLLLLLLLLVSWASRGEAAPDQDEIQRLPGLAKQPSFRQY
SGYLKSSGSKHLHYWFVESQKDPENSPVVLWLNGGPGCSSLDGLLTEHGP
FLVQPDGVTLEYNPYSWNLIANVLYLESPAGVGFSYSDDKFYATNDTEVA
QSNFEALQDFFRLFPEYKNNKLFLTGESYAGIYIPTLAVLVMQDPSMNLQ
GLAVGNGLSSYEQNDNSLVYFAYYHGLLGNRLWSSLQTHCCSQNKCNFYD
NKDLECVTNLQEVARIVGNSGLNIYNLYAPCAGGVPSHFRYEKDTVVVQD
LGNIFTRLPLKRMWHQALLRSGDKVRMDPPCTNTTAASTYLNNPYVRKAL
NIPEQLPQWDMCNFLVNLQYRRLYRSMNSQYLKLLSSQKYQILLYNGDVD
MACNFMGDEWFVDSLNQKMEVQRRPWLVKYGDSGEQIAGFVKEFSHIAFL
TIKGAGHMVPTDKPLAAFTMFSRFLNKQPY
Legend This sequence has been compared to family alignement (MSA)
red => minority aminoacid
blue => majority aminoacid
color intensity => conservation rate
title => sequence position(MSA position)aminoacid rate
Catalytic site
Catalytic site in the MSA

MIRAAPPPLFLLLLLLLLLVSWASRGEAAPDQDEIQRLPGLAKQPSFRQY
SGYLKSSGSKHLHYWFVESQKDPENSPVVLWLNGGPGCSSLDGLLTEHGP
FLVQPDGVTLEYNPYSWNLIANVLYLESPAGVGFSYSDDKFYATNDTEVA
QSNFEALQDFFRLFPEYKNNKLFLTGESYAGIYIPTLAVLVMQDPSMNLQ
GLAVGNGLSSYEQNDNSLVYFAYYHGLLGNRLWSSLQTHCCSQNKCNFYD
NKDLECVTNLQEVARIVGNSGLNIYNLYAPCAGGVPSHFRYEKDTVVVQD
LGNIFTRLPLKRMWHQALLRSGDKVRMDPPCTNTTAASTYLNNPYVRKAL
NIPEQLPQWDMCNFLVNLQYRRLYRSMNSQYLKLLSSQKYQILLYNGDVD
MACNFMGDEWFVDSLNQKMEVQRRPWLVKYGDSGEQIAGFVKEFSHIAFL
TIKGAGHMVPTDKPLAAFTMFSRFLNKQPY

no DNA




References
76 more
    Title: Structural and Kinetic Evidence of Aging after Organophosphate Inhibition of Human Cathepsin A
    Bouknight KD, Jurkouich KM, Compton JR, Khavrutskii IV, Guelta MA, Harvey SP, Legler PM
    Ref: Biochemical Pharmacology, :113980, 2020 : PubMed

            

    Title: Remodeling natural products: chemistry and serine hydrolase activity of a rocaglate-derived beta-lactone
    Lajkiewicz NJ, Cognetta AB, 3rd, Niphakis MJ, Cravatt BF, Porco JA, Jr.
    Ref: Journal of the American Chemical Society, 136:2659, 2014 : PubMed

            

    Title: Crystal structure of cathepsin A, a novel target for the treatment of cardiovascular diseases
    Schreuder HA, Liesum A, Kroll K, Bohnisch B, Buning C, Ruf S, Sadowski T
    Ref: Biochemical & Biophysical Research Communications, 445:451, 2014 : PubMed

            


Other Papers


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Mail to: Nicolas Lenfant, Thierry Hotelier, Yves Bourne, Pascale Marchot and Arnaud Chatonnet.
Please cite: Lenfant 2013 Nucleic.Acids.Res. or Marchot Chatonnet 2012 Prot.Pept Lett.
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