biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
enhanced validation
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
technique(s)
immunoblotting: 0.04-0.4 μg/mL, immunohistochemistry: 1:500-1:1000
immunogen sequence
ENSTSYEEAKNLLTKTKILAPAYFILGGNQSGEGCVITRDRKESLDVYELDAKQGRWYVVQTNYDRWKHPFFLDDRRTPAKMCLNRTSQENISFETMYDVLSTKPVLNKLTVYTTLIDVTKGQF
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... ASAH1(427)
General description
N-acylsphingosine amidohydrolase (ASAH1) gene is located on human chromosome 8p22-21.2. It is a 26.5kb long gene, which has 14 exons and 13 introns. This gene codes for a 55kDa protein with two subunits- 13kDa α and 40kDa β subunits. It is a glycoprotein and the two subunits are produced by auto-proteolytic cleavage. This protein is highly expressed in heart, lung, kidney and placenta and is expressed to a lesser extent in brain, liver, pancreas and skeletal muscle. ASAH1 is localized to lysosomes and human fibroblasts and macrophages secrete it extracellularly.
Immunogen
Acid ceramidase precursor recombinant protein epitope signature tag (PrEST)
Other Notes
Corresponding Antigen APREST70303
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Application
Anti-ASAH1 antibody is suitable for chromatin immunoprecipitation (ChIP) and reChIP.
Anti-ASAH1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Anti-ASAH1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem/physiol Actions
N-acylsphingosine amidohydrolase (ASAH1) converts ceramide to fatty acid and sphingosine. ASAH1 deficiency leads to Farber disease (FD) or Farber lipogranulomatosis, which is a lysosomal storage disease. It is characterized by pulmonary insufficiency, painful swelling of joints and tendons, and a shortened life-span, because of the accumulation of ceramide in tissues. It regulates adrenocortical steroidogenesis by maintaining the intracellular balance of ceramide, sphingosine and sphingosine-1-phosphate. These molecules in turn act as second messengers in protein kinase A/cAMP-dependent pathway mediated steroidogenesis. Expression of ASAH1 is found to be elevated in multiple tumor types, especially prostate cancer. Studies suggest that ASAH1 is a potential therapeutic target in chemoresistant and advanced prostate cancer types.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
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存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
此项目有
Luz Camacho et al.
Journal of lipid research, 54(5), 1207-1220 (2013-02-21)
Acid ceramidase (AC) catalyzes the hydrolysis of ceramide into sphingosine, in turn a substrate of sphingosine kinases that catalyze its conversion into the mitogenic sphingosine-1-phosphate. AC is expressed at high levels in several tumor types and has been proposed as
Justine Leclerc et al.
Oncogene, 38(8), 1282-1295 (2018-09-27)
Phenotypic plasticity and subsequent generation of intratumoral heterogeneity underly key traits in malignant melanoma such as drug resistance and metastasis. Melanoma plasticity promotes a switch between proliferative and invasive phenotypes characterized by different transcriptional programs of which MITF is a
Z Zhang et al.
Molecular genetics and metabolism, 70(4), 301-309 (2000-09-20)
Farber disease is an autosomal recessive disorder caused by lysosomal acid ceramidase (AC) deficiency. It commonly manifests during the first few months after birth with a unique triad of painful and progressive deformed joints, subcutaneous nodules, and progressive hoarseness. In
C M Li et al.
Genomics, 62(2), 223-231 (1999-12-28)
Acid ceramidase (AC) is the lysosomal enzyme that degrades ceramide into sphingosine and fatty acid. A deficiency in human AC activity leads to the lysosomal storage disorder, Farber disease (FD). The human AC gene (HGMW-approved symbol ASAH) was cloned and
J Koch et al.
The Journal of biological chemistry, 271(51), 33110-33115 (1996-12-20)
Human acid ceramidase ((AC) N-acylsphingosine amidohydrolase, EC 3.5. 1.23) hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Ceramide is an essential component of all sphingolipids and an important cell-signaling molecule. Moreover, an inherited deficiency of AC activity leads
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