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Anti-ARSB Antibody

品牌：Antibodies

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規(guī)格：100μg

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Anti-ARSB Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

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.