Anti-PSAP antibody (ab88251)

Overview

• Product nameAnti-PSAP antibodySee all PSAP primary antibodies ...
• Description
  Rabbit polyclonal to PSAP
• Tested applicationsICC/IF, WB more details
• Species reactivity
  Reacts with: Human
  Predicted to work with: Orangutan
• Immunogen

  Synthetic peptide conjugated to KLH derived from within residues 350 - 450 of Human PSAP.

  Read Abcam's proprietary immunogen policy

• Positive controlThis antibody gave a positive signal in the following human tissue lysates: Kidney; Testis.

Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: 0.02% Sodium Azide
  Constituents: 1% BSA, PBS, pH 7.4
• Concentration information loading...
• PurityImmunogen affinity purified
• Clonality Polyclonal
• IsotypeIgG
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Energy transfer pathways
  • Energy Metabolism

  • Signal Transduction
  • Metabolism
  • Energy Metabolism

  • Signal Transduction
  • Metabolism
  • Lipid metabolism

Applications

Target

• FunctionThe 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).
  Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). Saposin-C apparently acts by combining with the enzyme and acidic lipid to form an activated complex, rather than by solubilizing the substrate.
  Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
  Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 3.1.4.12).
• Involvement in diseaseDefects in PSAP are the cause of combined saposin deficiency (CSAPD) [MIM:611721]; 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) [MIM:249900]. 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) [MIM:610539]. 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) [MIM:611722]. AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease.
  Note=Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis).
• Sequence similaritiesContains 2 saposin A-type domains.
  Contains 4 saposin B-type domains.
• Post-translational
  modificationsThis precursor is proteolytically processed to 4 small peptides, which are similar to each other and are sphingolipid hydrolase activator proteins.
  N-linked glycans show a high degree of microheterogeneity.
  The one residue extended Saposin-B-Val is only found in 5% of the chains.
• Cellular localizationLysosome.

Target information above from: UniProt accession P07602 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 5660 Human
  • Omim: 176801 Human
  • SwissProt: P07602 Human
  • Unigene: 523004 Human

• Alternative names
  A1 activator antibodyCerebroside sulfate activator antibodyCo-beta-glucosidase antibody

  Component C antibodyCSAct antibodyDispersin antibodyGLBA antibodyGlucosylceramidase activator antibodyProactivator polypeptide antibodyProactivator polypeptide precursor antibodyProsaposin (sphingolipid activator protein 1) antibodyprosaposin (variant Gaucher disease and variant metachromatic leukodystrophy) antibodyProsaposin antibodyProtein A antibodyProtein C antibodyPSAP antibodySAP-1 antibodySAP-2 antibodySAP_HUMAN antibodySAP1 antibodySaposin A antibodySaposin B antibodySaposin B Val antibodySaposin C antibodySaposin D antibodySaposin-D antibodySaposins antibodySgp1 antibodySphingolipid activator protein 1 antibodySphingolipid activator protein 2 antibodySulfated glycoprotein 1 antibodySulfatide/GM1 activator antibody

  see all

Anti-PSAP antibody images

• 

  Western blot - PSAP antibody (ab88251)
  All lanes : Anti-PSAP antibody (ab88251) at 1 µg/ml
  
  Lane 1 : Kidney (Human) Tissue Lysate - adult normal tissue (ab30203)
  Lane 2 : Testis (Human) Tissue Lysate - adult normal tissue (ab30257)
  
  Lysates/proteins at 10 µg per lane.
  
  Secondary
  Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (HRP), pre-adsorbed (ab97080) at 1/5000 dilution
  developed using the ECL technique
  
  Performed under reducing conditions.
  
  Predicted band size : 58 kDa
  Observed band size : 55 kDa (why is the actual band size different from the predicted?)
  
  
  Exposure time : 20 minutes
• 

  Immunocytochemistry/ Immunofluorescence - PSAP antibody (ab88251)

  ICC/IF image of ab88251 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody ab88251 at 5µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit IgG (H+L)(ab96899) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.