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ABN468 Anti-ASAH1 Antibody

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ABN468
100 µg  
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      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, MWB, IHC, IFRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue NumberABN468
      DescriptionAnti-ASAH1 Antibody
      Alternate Names
      • ASAH1 Antibody
      • N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 1
      • acid ceramidase
      • hCG_28255
      Background InformationN-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.
      References
      Product Information
      FormatAffinity Purified
      Control
      • Mouse heart tissue lysate
      PresentationPurified rabbit polyclonal in buffer containing PBS with up to 0.1% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-ASAH1 Antibody is a highly specific rabbit polyclonal antibody, that targets ASAH1 & has been tested in western blotting, IHC & Immunofluorescence.
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      • Immunofluorescence
      Application NotesImmunohistochemistry 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.
      Biological Information
      ImmunogenKLH-conjugated linear peptide corresponding to human ASAH1.
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRabbit
      Species Reactivity
      • Human
      • Mouse
      Antibody TypePolyclonal Antibody
      Gene Symbol
      • ASAH1
      Purification MethodAntigen Affinity Purified
      UniProt Number
      Molecular Weight~52 kDa observed. Uncharacterized bands may appear in some lysate(s).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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.
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Numero di catalogo GTIN
      ABN468 04053252923487