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ASAH1 Antibody

Product Specifications

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.

NCBI Gene ID

427

Swiss Prot

Q13510

Accession Number

EAW63795

Host

Rabbit

Reactivity

Human, Mouse, Rat

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Homeostasis

Purification

ASAH1 Antibody is affinity chromatography purified via peptide column.

Positive Control

Cat. No. 1301 - Human Heart Tissue Lysate

Concentration

1 mg/mL

Homology

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

Buffer

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

Modification

None

Shipping Conditions

Blue Ice

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.

Product Datasheet

https://www.prosci-inc.com/?datasheet_sku=4741

Fragment

IgG

Specificity

Multiple isoforms of ASAH1 are known to exist.

Symbol

ASAH1

Positive Control 2

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

NCBI Official Name

N-acylsphingosine amidohydrolase (acid ceramidase) 1

NCBI Organism

Homo sapiens

Background Reference 01

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

Background Reference 02

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.

Background Reference 03

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.

Background Reference 04

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

Other Product Names

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

Tested Applications

ELISA, WB, IHC-P, IF

Protein ID

119584199

Physical Properties

Liquid

Available Sizes

Curated Selection

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