Anti-ARSB Antibody (CAB16765)
- SKU:
- CAB16765
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-ARSB Antibody |
| 抗体コード: | CAB16765 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | IHC IF |
| 反応性: | Human, Mouse |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 404-533 of human ARSB (NP_000037.2). |
| 申し込み: | IHC IF |
| 推奨希釈: | IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse |
| ポジティブサンプル: |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 404-533 of human ARSB (NP_000037.2). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | PCPR NSMA PAKD DSSL PEYS AFNT SVHA AIRH GNWK LLTG YPGC GYWF PPPS QYNV SEIP SSDP PTKT LWLF DIDR DPEE RHDL SREY PHIV TKLL SRLQ FYHK HSVP VYFP AQDP RCDP KATG VWGP WM |
| 遺伝子ID: | 411 |
| Uniprot: | P15848 |
| セルラーロケーション: | Cell surface, Lysosome |
| 計算された分子量: | 45kDa/59kDa |
| 観察された分子量: | Refer to figures |
 | Immunohistochemistry - ARSB Rabbit pAb (CAB16765) |
| 同義語: | ARSB, ASB, G4S, MPS6 |
| バックグラウンド: | 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 targeted 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. |
| 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 |