Anti-ASAH1 Antibody

半岛bd体育手机客户端 说明

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.

免疫原

KLH-conjugated linear peptide corresponding to human ASAH1.

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 ?g/mL from a representative lot detected ASAH1 in human heart cells.

质量

Evaluated by Western Blot in mouse heart tissue lysate.

Western Blot Analysis: 2 ?g/mL of this antibody detected ASAH1 in 15 ?g of mouse heart tissue lysate.

目标描述

~52 kDa observed. Uncharacterized bands may appear in some lysate(s).

外形

Purified rabbit polyclonal in buffer containing PBS with up to 0.1% sodium azide.
Antigen Affinity Purified

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

分析说明

Control
Mouse heart tissue lysate

其他说明

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

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.

基本信息

eCl@ss	32160702
NACRES	NA.41

半岛bd体育手机客户端 性质

生物来源	rabbit
质量水平	100
抗体形式	affinity isolated antibody
antibody product type	primary antibodies
克隆	polyclonal
纯化方式	affinity chromatography
species reactivity	human, mouse
technique(s)	immunofluorescence: suitable immunohistochemistry: suitable western blot: suitable
UniProt登记号	D3DSQ1
运输	wet ice
Gene Information	human ... ASAH1(427)

安全信息

储存分类代码	10 - Combustible liquids
WGK	WGK 2
闪点(F)	Not applicable
闪点(C)	Not applicable