Rabbit Anti-Protective protein/Cathepsin A antibody

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Cathepsin A; CTSA; BETA GALACTOSIDASE PROTECTIVE PROTEIN; Carboxypeptidase C; Glactosialidosis; GLB2; Goldberg Syndrome; GSL; Lysosomal protective protein; NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE; NGBE; PPCA; PPGB; Protective protein for beta ga

Cat:

SL6040R

Species Reactivity：

Human,Mouse,Rat,(predicted: Dog,Pig,Cow,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human Protective protein:401-480/480

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Sample: Spleen (Mouse) Lysate at 40 ugPrimary: Anti-Protective protein (SL6040R) at 1/300 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 51 kDObserved band size: 51 kDSample: 293T Cell (Human) Lysate at 30 ugPrimary: Anti-Protective protein (SL6040R) at 1/300 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 51 kDObserved band size: 51 kD Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Protective protein,Cathepsin A) Polyclonal Antibody, Unconjugated (SL6040R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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Lysosomal protective protein/cathepsin A (PPCA) is a lysosomal serine carboxypeptidase that forms an intralysosomal enzyme complex with ?galactosidase and neuraminidase (NEU1). PPCA is synthesized as a 54 kDa precursor/zymogen, and proteolytically cleaved in the lysosome into a catalytically active 32 and 20 kDa two chain enzyme. The enzyme has cathepsin A activity at acidic pH but maintains also a deamidase/esterase activity at neutral pH. Furthermore, the human enzyme, purified from platelets and lymphocytes, has been shown to function on the inactivation of selected neuropeptides, like substance P, oxytocin, and endothelin I. The autosomal recessive genetic deficiency of PPCA causes galactosialidosis, a neurodegenerative lysosomal storage disorder, resulting in the secondary deficiencies of ?galactosidase and NEU1.

Function:
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.

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

Subcellular Location:
Lysosome.

DISEASE:
Defects in CTSA are the cause of 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.

Similarity:
Belongs to the peptidase S10 family.

SWISS:
P10619

Gene ID:
5476

Database links:

Entrez Gene: 5476 Human

Entrez Gene: 19025 Mouse

Omim: 613111 Human

SwissProt: P10619 Human

SwissProt: P16675 Mouse

Unigene: 609336 Human

Unigene: 359633 Mouse

Unigene: 474586 Mouse

Picture

Sample:
293T Cell (Human) Lysate at 30 ug
Primary: Anti-Protective protein (SL6040R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 51 kD
Observed band size: 51 kD

Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Protective protein,Cathepsin A) Polyclonal Antibody, Unconjugated (SL6040R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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