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Anti-ASAH1 Antibody

产品编号:4105113
规格:from rabbit, purified by affinity chromatography
包装规格:100 μG
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
4105113100 μG6190
产品别名

Anti-ASAH1 Antibody

ASAH1 Antibody, N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 1, acid ceramidase, hCG_28255

基本信息
eCl@ss
32160702
NACRES
NA.41
General description【一般描述】
N-acylsphingosine amidohydrolase (Acid ceramidase) 1, or ASAH1 is part of a family of hydrolyases, localized in the lysosome, that catalyze the degradation of ceramide into sphingosine (SPH) and free fatty acid. Ceraminde is a subgroup of sphingolipids and sphingolipids are important membrane lipids in all cells. Sphingolipids are being recognized today too as important second messengers in various signaling transduction pathways, principally via the release or use of free sphingosine. Because ceramide degradation is the only source of cellular SPH, these enzymes are not only important for limiting ceramide-mediated signaling but also for controlling the cellular functions of SPH and SPH-1 phosphate enzymes. Build up excess ceramides can have damaging effects on cells and indeed, ceramide addition is a potent inducer of apoptosis is most cell types. Defects in ASAH1 gene cause a condition called Farber lipogranulomatosis, also known as Farber disease (FD). This sphingolipid disease is characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, marked accumulation of ceramide in lysosomes, and death by three years of age. Additionally, spinal muscular atrophy is also associated with defects in the ASAH1 protein which eventually results in death at an early age from respiratory failure. EMD-Millipore’s anti-ASAH1 rabbit polyclonal antibody has been tested in western blot on mouse heart tissue lysates, and by immunohistochemistry on human heart tissue, and by fluorescent immunocytochemistry on human heart cells in culture.
Immunogen【免疫原】
KLH-conjugated linear peptide corresponding to human ASAH1.
Application【应用】
Research Category
Neuroscience
Research Sub Category
Developmental Neuroscience
Anti-ASAH1 Antibody is a highly specific rabbit polyclonal antibody, that targets ASAH1 & has been tested in western blotting, IHC & Immunofluorescence.
Immunohistochemistry Analysis: A 1:1,000 dilution from a representative lot detected ASAH1 in human cardiac myocytes and kidney tissues.

Immunofluorescence Analysis: 20 µg/mL from a representative lot detected ASAH1 in human heart cells.
Quality【质量】
Evaluated by Western Blot in mouse heart tissue lysate.

Western Blot Analysis: 2 µg/mL of this antibody detected ASAH1 in 15 µg of mouse heart tissue lysate.
Physical form【外形】
Purified rabbit polyclonal in buffer containing PBS with up to 0.1% sodium azide.
Antigen Affinity Purified
Analysis Note【分析说明】
Control
Mouse heart tissue lysate
Other Notes【其他说明】
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
产品性质
biological source【生物来源】
rabbit
Quality Level【质量水平】
100
antibody form【抗体形式】
affinity isolated antibody
antibody product type
primary antibodies
clone【克隆】
polyclonal
purified by【纯化方式】
affinity chromatography
species reactivity
human, mouse
technique(s)
immunofluorescence: suitable
immunohistochemistry: suitable
western blot: suitable
UniProt accession no.【UniProt登记号】
D3DSQ1
shipped in【运输】
wet ice
Gene Information
human ... ASAH1(427)
产品说明
Target description【目标描述】
~52 kDa observed. Uncharacterized bands may appear in some lysate(s).
Storage and Stability【储存及稳定性】
Stable for 1 year at 2-8°C from date of receipt.
Disclaimer【免责声明】
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.
安全信息
Storage Class Code【储存分类代码】
10 - Combustible liquids
WGK
WGK 2
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