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Antibodies
Anti-ARSB Antibody
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产品名称:
Anti-ARSB Antibody
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简单介绍
Anti-ARSB
Antibody
Anti-ARSB Antibody
的详细介绍
Overview
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.
Target
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 Location:
Lysosome. Cell surface.
Database Links:
Entrez Gene: 411?Human
Omim: 611542?Human
SwissProt: P15848?Human
Unigene: 149103?Human
Unigene: 604199?Human
Synonyms:
ARSB Antibody
ARSB_HUMAN Antibody
Arylsulfatase B Antibody
ArylsulfataseB Antibody
ASB Antibody
G4S Antibody
MPS6 Antibody
N acetylgalactosamine 4 sulfatase Antibody
N-acetylgalactosamine-4-sulfatase Antibody
Information:
Target information shown above is from the UniProt Consortium.
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