ARSB Antibody (PACO25064)
- SKU:
- PACO25064
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | ARSB Antibody (PACO25064) |
抗体コード: | PACO25064 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:1000-1:5000 |
反応性: | Human, Mouse |
免疫原: | Recombinant Human Arylsulfatase B protein (37-533AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot All lanes: ARSB antibody at 2µg/ml + Mouse brain tissue Secondary Goat polyclonal to rabbit lgG at 1/15000 dilution Predicted band size: 60, 46 kDa Observed band size: 40 kDa . |
シノニム: | Arylsulfatase B (ASB) (EC 3.1.6.12) (N-acetylgalactosamine-4-sulfatase) (G4S), ARSB |
UniProt Protein Function: | ARSB: Defects in ARSB are the cause of mucopolysaccharidosis type 6 (MPS6); also known as Maroteaux-Lamy syndrome. MPS6 is 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. Arylsulfatase B activity is defective in multiple sulfatase deficiency (MSD). 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. Arylsulfatase B activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSB at residue Cys- 91 that is not converted to 3-oxoalanine. Belongs to the sulfatase family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Hydrolase; EC 3.1.6.12; Glycan Metabolism - glycosaminoglycan degradation Chromosomal Location of Human Ortholog: 5q14.1 Cellular Component: cell surface; endoplasmic reticulum lumen; lysosomal lumen; lysosome Molecular Function:arylsulfatase activity; N-acetylgalactosamine-4-sulfatase activity Biological Process: chondroitin sulfate catabolic process; glycosphingolipid metabolic process; lysosomal transport; lysosome organization and biogenesis; post-translational protein modification Disease: Mucopolysaccharidosis Type Vi |
NCBI Summary: | Arylsulfatase B encoded by this gene belongs to the sulfatase family. The arylsulfatase B homodimer hydrolyzes sulfate groups of N-Acetyl-D-galactosamine, chondriotin sulfate, and dermatan sulfate. The protein is targetted to the lysozyme. Mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Two alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | P15848 |
NCBI GenInfo Identifier: | 114223 |
NCBI Gene ID: | 411 |
NCBI Accession: | P15848.1 |
UniProt Secondary Accession: | P15848,Q8N322, Q9UDI9, B2RC20, |
UniProt Related Accession: | P15848 |
Molecular Weight: | 45,996 Da |
NCBI Full Name: | Arylsulfatase B |
NCBI Synonym Full Names: | arylsulfatase B |
NCBI Official Symbol: | ARSB |
NCBI Official Synonym Symbols: | ASB; G4S; MPS6 |
NCBI Protein Information: | arylsulfatase B |
UniProt Protein Name: | Arylsulfatase B |
UniProt Synonym Protein Names: | N-acetylgalactosamine-4-sulfatase; G4S |
Protein Family: | Arylsulfatase |
UniProt Gene Name: | ARSB |
UniProt Entry Name: | ARSB_HUMAN |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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