产品名称
Glucuronidase β Rabbit Polyclonal Antibody
别名
GUSB; Beta-glucuronidase; Beta-G1
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2990
Human Swissprot No.
P08236
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P08236/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=110006
Mouse Swissprot No.
P12265
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P12265
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=24434
Rat Swissprot Link
http://www.uniprot.org/uniprot/P06760
免疫原
The antiserum was produced against synthesized peptide derived from human GUSB. AA range:321-370
特异性
Glucuronidase β Polyclonal Antibody detects endogenous levels of Glucuronidase β protein.
稀释度
IHC-p: 100-300.WB 1:500 - 1:2000. ELISA: 1:10000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014],
组织表达
Colon,Fibroblast,Liver,Placenta,Plasma,
信号通路
Pentose and glucuronate interconversions;Starch and sucrose metabolism;Glycosaminoglycan degradation;Porphyrin and chlorophyll metabolism;Drug metabolism;Lysosome;
功能
catalytic activity:A beta-D-glucuronoside + H(2)O = D-glucuronate + an alcohol.,disease:Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) [MIM:253220]; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment.,disease:Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis [MIM:236750]. Hydrops fetalis is a generalized edema of the fetus with fluid accumulation in the body cavities.,enzyme regulation:Inhibited by L-aspartic acid.,function:Plays an important role in the degradation of dermatan and keratan sulfates.,PTM:N-linked glycosylated with 3 to 4 oligosaccharide chains.,similarity:Belongs to the glycosyl hydrolase 2 family.,subunit:Homotetramer.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
