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Beta glucuronidase Monoclonal Antibody

Applications

  • WB
  • FCM
  • IHC-P
  • IF(ICC)

Reactivity

  • Human
Overview
Catalog # bsm-54726R
Product Name Beta glucuronidase Monoclonal Antibody
Applications WB, FCM, IHC-P, IF(ICC)
Reactivity Human
Specifications
Conjugation Unconjugated
Host Rabbit
Source Synthetic Peptide within Human beta glucuronidase
Clonality Monoclonal
Clone # 3H4
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 1xTBS (pH7.4), 1%BSA, 40%Glycerol and 0.05% Sodium Azide.
Storage Condition Store at -20°C for 12 months.
Target
Gene ID 2990
Swiss Prot P08236
Subcellular location Lysosome
Synonyms Ac2-223 antibody, asd antibody, Beta G1 antibody, Beta glucuronidase antibody, Beta-G1 antibody, Beta-glucuronidase antibody, BG antibody, BGLR antibody, BGLR_HUMAN antibody, FLJ39445 antibody, Glucuronidase beta antibody, Gur antibody, Gus antibody, Gus-r antibody, Gus-s antibody, Gus-t antibody, Gus-u antibody, GUSB antibody, Gut antibody, MPS7 antibody
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. 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.
Application Dilution
WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400
IF(ICC) 1:50-200