Metabolism Antibodies 2

Anti-PSAP Antibody (CAB1819)

(No reviews yet) Write a Review
SKU:
CAB1819
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Metabolism

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-PSAP Antibody
Antibody SKU:CAB1819
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 60-310 of human PSAP (NP_002769.1).
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:A-549, SKOV3, 293T
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 60-310 of human PSAP (NP_002769.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:SLPC DICK DVVT AAGD MLKD NATE EEIL VYLE KTCD WLPK PNMS ASCK EIVD SYLP VILD IIKG EMSR PGEV CSAL NLCE SLQK HLAE LNHQ KQLE SNKI PELD MTEV VAPF MANI PLLL YPQD GPRS KPQP KDNG DVCQ DCIQ MVTD IQTA VRTN STFV QALV EHVK EECD RLGP GMAD ICKN YISQ YSEI AIQM MMHM QPKE ICAL VGFC DEVK EMPM QTLV PAKV ASKN VIPA LELV EPIK KHEV PAK
Gene ID:5660
Uniprot:P07602
Cellular Location:Lysosome, Secreted
Calculated MW:58kDa
Observed MW:58kDa
Synonyms:PSAP, GLBA, SAP1
Background:This gene encodes a highly conserved preproprotein that is proteolytically processed to generate four main cleavage products including saposins A, B, C, and D. Each domain of the precursor protein is approximately 80 amino acid residues long with nearly identical placement of cysteine residues and glycosylation sites. Saposins A-D localize primarily to the lysosomal compartment where they facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. The precursor protein exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. Mutations in this gene have been associated with Gaucher disease and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed.
UniProt Protein Function:PSAP: The lysosomal degradation of sphingolipids takes place by the sequential action of specific hydrolases. Some of these enzymes require specific low-molecular mass, non-enzymic proteins: the sphingolipids activator proteins (coproteins). Defects in PSAP are the cause of combined saposin deficiency (CSAPD); also known as prosaposin deficiency. CSAPD is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement. Defects in PSAP saposin-B region are the cause of leukodystrophy metachromatic due to saposin-B deficiency (MLD- SAPB). MLD-SAPB is an atypical form of metachromatic leukodystrophy. It is characterized by tissue accumulation of cerebroside-3-sulfate, demyelination, periventricular white matter abnormalities, peripheral neuropathy. Additional neurological features include dysarthria, ataxic gait, psychomotr regression, seizures, cognitive decline and spastic quadriparesis. Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD). Affected individuals have marked glucosylceramide accumulation in the spleen without having a deficiency of glucosylceramide-beta glucosidase characteristic of classic Gaucher disease, a lysosomal storage disorder. Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD). AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease. Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis). 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Chromosomal Location of Human Ortholog: 10q21-q22

Cellular Component: cytoplasm; extracellular region; extracellular space; integral to membrane; lysosomal lumen; lysosomal membrane; mitochondrion

Molecular Function:enzyme activator activity; G-protein-coupled receptor binding; lipid binding; protein binding

Biological Process: blood coagulation; G-protein signaling, adenylate cyclase inhibiting pathway; glycosphingolipid metabolic process; lipid transport; platelet activation; platelet degranulation; positive regulation of catalytic activity; positive regulation of MAPKKK cascade; regulation of lipid metabolic process; sphingolipid metabolic process

Disease: Combined Saposin Deficiency; Gaucher Disease, Atypical, Due To Saposin C Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency

NCBI Summary:This gene encodes a highly conserved preproprotein that is proteolytically processed to generate four main cleavage products including saposins A, B, C, and D. Each domain of the precursor protein is approximately 80 amino acid residues long with nearly identical placement of cysteine residues and glycosylation sites. Saposins A-D localize primarily to the lysosomal compartment where they facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. The precursor protein exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. Mutations in this gene have been associated with Gaucher disease and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Feb 2016]
UniProt Code:P07602
NCBI GenInfo Identifier:134218
NCBI Gene ID:5660
NCBI Accession:P07602.2
UniProt Secondary Accession:P07602,P07292, P15793, P78538, P78541, P78546, P78547 P78558, Q53Y86, Q6IBQ6, Q92739, Q92740,
UniProt Related Accession:P07602
Molecular Weight:58,484 Da
NCBI Full Name:Prosaposin
NCBI Synonym Full Names:prosaposin
NCBI Official Symbol:PSAP  
NCBI Official Synonym Symbols:GLBA; SAP1  
NCBI Protein Information:prosaposin
UniProt Protein Name:Prosaposin
UniProt Synonym Protein Names:Proactivator polypeptide
Protein Family:Prosaposin
UniProt Gene Name:PSAP  
UniProt Entry Name:SAP_HUMAN
Anti-PSAP Antibody (CAB1819)Western blot analysis of extracts of various cell lines, using PSAP antibody (CAB1819) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 5s.
Anti-PSAP Antibody (CAB1819)Immunohistochemistry of paraffin-embedded rat brain using PSAP antibody (CAB1819) at dilution of 1:200 (40x lens).
Anti-PSAP Antibody (CAB1819)Immunohistochemistry of paraffin-embedded rat spleen using PSAP antibody (CAB1819) at dilution of 1:200 (40x lens).
Anti-PSAP Antibody (CAB1819)Immunohistochemistry of paraffin-embedded mouse brain using PSAP antibody (CAB1819) at dilution of 1:200 (40x lens).
Anti-PSAP Antibody (CAB1819)Immunofluorescence analysis of MCF-7 cells using PSAP antibody (CAB1819). Blue: DAPI for nuclear staining.
View AllClose

0 Reviews

View AllClose

Related Products

PSAP Polyclonal Antibody

PSAP Polyclonal Antibody

OverviewProduct Name:PSAP Polyclonal AntibodyProduct Code:CAB21761Reactivity:Human, Mouse, RatApplications:Western blotting, Immu