Datasheet

ASAH1 Antibody
CATALOG NUMBER: 4741

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Specifications
Properties
Additional Info
Background

Specifications

SPECIES REACTIVITY:Human, Mouse, Rat
HOMOLOGY:Predicted species reactivity based on immunogen sequence: Bovine: (88%)
TESTED APPLICATIONS:ELISA, IF, IHC-P, WB
APPLICATIONS:ASAH1 antibody can be used for detection of ASAH1 by Western blot at 1 and 2 μg/mL. Antibody can also be used for immunohistochemistry starting at 2.5 μg/mL. For immunofluorescence start at 20 μg/mL.
USER NOTE:Optimal dilutions for each application to be determined by the researcher.
POSITIVE CONTROL:1) Cat. No. 1301 - Human Heart Tissue Lysate
 2) Cat. No. 10-501 - Human Heart Tissue Slide
PREDICTED MOLECULAR WEIGHT:Predicted: 45 kDa

Observed: 49 kDa
SPECIFICITY:Multiple isoforms of ASAH1 are known to exist.
IMMUNOGEN:ASAH1 antibody was raised against a 16 amino acid synthetic peptide near the carboxy terminus of the human ASAH1.

The immunogen is located within amino acids 240 - 290 of ASAH1.
HOST SPECIES:Rabbit

Properties

PURIFICATION:ASAH1 Antibody is affinity chromatography purified via peptide column.
PHYSICAL STATE:Liquid
BUFFER:ASAH1 Antibody is supplied in PBS containing 0.02% sodium azide.
CONCENTRATION:1 mg/mL
STORAGE CONDITIONS:ASAH1 antibody can be stored at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
CLONALITY:Polyclonal
ISOTYPE:IgG
CONJUGATE:Unconjugated

Additional Info

ALTERNATE NAMES:ASAH1 Antibody: AC, PHP, ASAH, PHP32, ACDase, SMAPME, HSD-33, HSD33, Acid ceramidase, Acylsphingosine deacylase, AC
ACCESSION NO.:EAW63795
PROTEIN GI NO.:119584199
OFFICIAL SYMBOL:ASAH1
GENE ID:427

Background

BACKGROUND:ASAH1 Antibody: Sphingolipids are hydrolyzed by ceramidases to yield sphingosine and fatty acids. These ceramidases are classified according to the pH range that supports their optimal activity. ASAH1 is an acid ceramidase and key regulator of ceramide metabolism. Mutations in this gene results in Farber Lipogranulomatosis, a fatal human genetic disorder that results in the painful swelling of the joints and tendons and pulminary insufficiency, while a complete knockout of its expression is lethal in mice. Recent studies have shown elevated levels of ASAH1 in Alzheimer's disease (AD) patients correlating with a reduction in sphingomyelin and elevation of ceramide. Pretreatment of cultured neurons with recombinant AHAH1 prevented the cells from undergoing A-beta (Ab)-induced apoptosis.
REFERENCES: 1) Nilsson A and Duan RD. Alkaline sphingomyelinases and ceramidases of the gastrointestinal tract. Chem. Phys. Lipids 1999; 102:97-105.
2) Koch J, Gartner S, Li CM, et al. Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification of the first molecular lesion causing Farber’s disease. J. Biol. Chem. 1996; 271:33110-5.
3) Li CM, Park JH, Simonaro CM, et al. Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics 2002; 79:218-24.
4) He X, Huang Y, Li B, et al. Deregulation of sphingolipid metabolism in Alzheimer’s disease. Neurobiol. Aging 2010; 31:398-408.

For Research Use Only