beta glucuronidase Antibody
Cat#:PL0306295
Isotype:Rabbit Polyclonal IgG
Immunogen:Peptide derived from human GUSB/beta glucuronidase.
Purification: Immunogen affinity purified
Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)
Background:
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.
Alternate Names:
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.
Specificity Reaction:Hu, Mo, Rat, Chi, Dog, Pig, Cow
Application Notes:WB: 1:100-500 E: 1:500-1000 IP: 1:20-100 IHC: 1:100-500 IF: 1:100-500
Storage :
Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.
This product for research use only, not for use in humans, therapeutic or diagnostic procedures.
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