Beta glucuronidase Monoclonal Antibody


Beta glucuronidase Monoclonal Antibody

  • Size: 100ul
  • Application: WB, FCM, IHC-P, IF(ICC)
  • Reactivity: Human
  • Predicted Reactivity:

  • Datasheet      Tech Support


Catalog# bsm-54726R

Size:100ul Datasheet


 SizePrice
 100ul ₹ 50828

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IMAGES






PRODUCT DETAILS






SPECIFICATIONS

PRODUCT NAME

Beta glucuronidase Monoclonal Antibody

CONJUGATION

Unconjugated

HOST

Rabbit

SOURCE

Synthetic Peptide within Human beta glucuronidase

IMMUNOGEN RANGE

CLONALITY

Monoclonal

ISOTYPE

IgG

CONCENTRATION

1ug/ul

PURIFICATION

Purified by Protein A.

STORAGE BUFFER

Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide.

STORAGE CONDITION

Store at -20°C for 12 months..


TARGET

GENE ID

2990

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(1:300-1000), FCM(1:20-100), IHC-P(1:200-400), IF(ICC)(1:50-200)












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