ASAH1 Antilichaam

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.

427

Q13510

EAW63795

Human, Mouse, Rat

Unconjugated

Primary Antibodies

Homeostasis

ASAH1 Antibody is affinity chromatography purified via peptide column.

Cat. No. 1301 - Human Heart Tissue Lysate

1 mg/mL

Predicted species reactivity based on immunogen sequence: Bovine: (88%)

ASAH1 Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

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.

IgG

Multiple isoforms of ASAH1 are known to exist.

ASAH1

Cat. No. 10-501 - Human Heart Tissue Slide

N-acylsphingosine amidohydrolase (acid ceramidase) 1

Homo sapiens

Nilsson A and Duan RD. Alkaline sphingomyelinases and ceramidases of the gastrointestinal tract. Chem. Phys. Lipids 1999; 102:97-105.

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.

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.

He X, Huang Y, Li B, et al. Deregulation of sphingolipid metabolism in Alzheimer's disease. Neurobiol. Aging 2010; 31:398-408.

ASAH1 Antibody: AC, PHP, ASAH, PHP32, ACDase, SMAPME, HSD-33, HSD33, Acid ceramidase, Acylsphingosine deacylase, AC

ELISA, WB, IHC-P, IF

119584199

Liquid