Product Name	beta glucuronidase
Chinese Name	β葡萄糖醛酸苷酶抗体
Alias	asd; Beta G1; Beta glucuronidase; Beta-G1; Beta-glucuronidase; BG; BGLR; BGLR_HUMAN; Glucuronidase beta; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; GUSB; Gut; MPS7; Ac2-223.
Research Area	Cell biology  immunology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	69kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human GUSB/beta glucuronidase: 589-651/651
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) ; 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. Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Function: Plays an important role in the degradation of dermatan and keratan sulfates. Subunit: Homotetramer. Subcellular Location: Lysosome. Post-translational modifications: N-linked glycosylated with 3 to 4 oligosaccharide chains. 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. Note=Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Similarity: Belongs to the glycosyl hydrolase 2 family. SWISS: P08236 Gene ID: 2990 Database links: Entrez Gene: 2990 Human Omim: 611499 Human SwissProt: P08236 Human Unigene: 255230 Human
Product Picture	Sample: Intestine (Mouse) Lysate at 40 ug Primary: Anti-beta glucuronidase (SL7980R) at 1/300 dilution Secondary: HRP conjugated Goat-Anti-rabbit IgG (SL0295G-HRP) at 1/5000 dilution Predicted band size: 69 kD Observed band size: 69 kD

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