Cell Biology Antibodies 4

Anti-ARSB Antibody (CAB14231)

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SKU:
CAB14231
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-ARSB Antibody
Antibody SKU:CAB14231
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 404-533 of human ARSB (NP_000037.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:U-87MG
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 404-533 of human ARSB (NP_000037.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence: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
Gene ID:411
Uniprot:P15848
Cellular Location:Cell surface, Lysosome
Calculated MW:45kDa/59kDa
Observed MW:60kDa
Synonyms:ARSB, ASB, G4S, MPS6
Background: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
Anti-ARSB Antibody (CAB14231)Western blot analysis of extracts of U-87MG cells, using ARSB antibody (CAB14231) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
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