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 Anti-ARSB Antibody |产品详情|进口榴莲视频免费观看采购网





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    Anti-ARSB Antibody
    品牌:Antibodies
    货号:
    规格:100µg
    货期:

    Anti-ARSB Antibody

    商品详情 参考文献 相关资料
    Name: Anti-ARSB Antibody
    See all ARSB primary antibodies
    Description: Goat polyclonal antibody to ARSB.
    Applications: ELISA, WB, IHC
    Reactivity: Human
    Immunogen: Synthetic peptide corresponding to Human ARSB (internal region).
    Sequence: C-KLARGHTNGTKPLD
    Host: Goat
    Clonality: Polyclonal
    Isotype: IgG
    Conjugate: Unconjugated
    Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
    Concentration: 100 μg at 0.5 mg/ml.
    Product Form: Liquid
    Formulation: Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
    Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
    Function: Removes sulfate groups from chondroitin-4-sulfate (C4S) and regulates its degradation (PubMed:19306108). Involved in the regulation of cell adhesion, cell migration and invasion in colonic epithelium (PubMed:19306108). In the central nervous system, is a regulator of neurite outgrowth and neuronal plasticity, acting through the control of sulfate glycosaminoglycans and neurocan levels (By similarity).
    Involvement in Disease: Mucopolysaccharidosis 6: An autosomal recessive lysosomal storage disease characterized by intracellular accumulation of dermatan sulfate. Clinical features can include abnormal growth, short stature, stiff joints, skeletal malformations, corneal clouding, hepatosplenomegaly, and cardiac abnormalities. A wide variation in clinical severity is observed.

    Multiple sulfatase deficiency: A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.
    Sequence Similarities: Belongs to the sulfatase family.
    Post-Translational Modification: The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).
    Cellular locations: Lysosome. Cell surface.
    Database Links:
  1. Entrez Gene: 411?Human
  2. Omim: 611542?Human
  3. SwissProt: P15848?Human
  4. Unigene: 149103?Human
  5. Unigene: 604199?Human
  6. Synonyms:
  7. ARSB Antibody
  8. ARSB_HUMAN Antibody
  9. Arylsulfatase B Antibody
  10. ArylsulfataseB Antibody
  11. ASB Antibody
  12. G4S Antibody
  13. MPS6 Antibody
  14. N acetylgalactosamine 4 sulfatase Antibody
  15. N-acetylgalactosamine-4-sulfatase Antibody
  16. Information: Target information shown above is from the UniProt Consortium.
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