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Protein Page:
CTSA (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
CTSA 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. Defects in CTSA are the cause of galactosialidosis (GSL). 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. Belongs to the peptidase S10 family. Note: This description may include information from UniProtKB.
Protein type: Endoplasmic reticulum; Protease; EC 3.4.16.5; Mitochondrial
Cellular Component: lysosomal lumen; intracellular membrane-bound organelle; membrane; endoplasmic reticulum; lysosome; nucleolus; nucleus
Molecular Function: serine carboxypeptidase activity; carboxypeptidase activity; enzyme activator activity
Biological Process: positive regulation of catalytic activity; intracellular protein transport; sphingolipid metabolic process; glycosphingolipid metabolic process; proteolysis
Reference #:  P10619 (UniProtKB)
Alt. Names/Synonyms: beta-galactosidase 2; beta-galactosidase protective protein; Carboxypeptidase C; Carboxypeptidase L; Cathepsin A; CTSA; GLB2; GSL; Lysosomal protective protein; Lysosomal protective protein 20 kDa chain; Lysosomal protective protein 32 kDa chain; NGBE; PPCA; PPGB; Protective protein cathepsin A; Protective protein for beta-galactosidase
Gene Symbols: CTSA
Molecular weight: 54,466 Da
Basal Isoelectric point: 6.16  Predict pI for various phosphorylation states
Select Structure to View Below

CTSA

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 Q36 APDQDEIQRLPGLAK
0 2 K55-m3 RQYSGYLkGSGSKHL
0 1 T295-p HFRYEKDtVVVQDLG
0 1 K429-ub QRRPWLVkyGDSGEQ
0 1 Y430-p RRPWLVkyGDSGEQI
0 1 T469-p DKPLAAFtMFsRFLN
0 1 S472-p LAAFtMFsRFLNKQP
  mouse

 
D31-ca APDQDEIdCLPGLAK
R50 RQYSGYLRASDSKHF
T289 GRHRYEDTLVVQDFG
D423 QRRPWLVDYGESGEQ
Y424 RRPWLVDYGESGEQV
T463 DKPRAAFTMFSRFLN
S466 RAAFTMFSRFLNKEP
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