ABN468
Anti-ASAH1 Antibody
from rabbit, purified by affinity chromatography
Synonyme(s) :
ASAH1 Antibody, N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 1, acid ceramidase, hCG_28255
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About This Item
Code UNSPSC :
12352203
eCl@ss :
32160702
Nomenclature NACRES :
NA.41
Produits recommandés
Source biologique
rabbit
Niveau de qualité
Forme d'anticorps
affinity isolated antibody
Type de produit anticorps
primary antibodies
Clone
polyclonal
Produit purifié par
affinity chromatography
Espèces réactives
mouse, human
Technique(s)
immunofluorescence: suitable
immunohistochemistry: suitable
western blot: suitable
Numéro d'accès UniProt
Conditions d'expédition
wet ice
Modification post-traductionnelle de la cible
unmodified
Informations sur le gène
human ... ASAH1(427)
Description générale
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.
Immunogène
KLH-conjugated linear peptide corresponding to human ASAH1.
Application
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.
Immunofluorescence Analysis: 20 µg/mL from a representative lot detected ASAH1 in human heart cells.
Research Category
Neuroscience
Neuroscience
Research Sub Category
Developmental Neuroscience
Developmental Neuroscience
Qualité
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.
Western Blot Analysis: 2 µg/mL of this antibody detected ASAH1 in 15 µg of mouse heart tissue lysate.
Description de la cible
~52 kDa observed. Uncharacterized bands may appear in some lysate(s).
Forme physique
Antigen Affinity Purified
Purified rabbit polyclonal in buffer containing PBS with up to 0.1% sodium azide.
Stockage et stabilité
Stable for 1 year at 2-8°C from date of receipt.
Remarque sur l'analyse
Control
Mouse heart tissue lysate
Mouse heart tissue lysate
Autres remarques
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Clause de non-responsabilité
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.
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Code de la classe de stockage
10 - Combustible liquids
Classe de danger pour l'eau (WGK)
WGK 2
Point d'éclair (°F)
Not applicable
Point d'éclair (°C)
Not applicable
Certificats d'analyse (COA)
Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".
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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.
Diego Iacono et al.
Neurobiology of disease, 174, 105862-105862 (2022-09-17)
Krabbe Disease (KD) is an autosomal recessive disorder that results from loss-of-function mutations in the GALC gene, which encodes lysosomal enzyme galactosylceramidase (GALC). Functional deficiency of GALC is toxic to myelin-producing cells, which leads to progressive demyelination in both the
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