Cell Biology Antibodies 9

Anti-GUSB Antibody (CAB5813)

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SKU:
CAB5813
Product Type:
Antibody
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-GUSB Antibody
Antibody SKU:CAB5813
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 382-651 of human GUSB (NP_000172.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Mouse
Positive Samples:Mouse skeletal muscle
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 382-651 of human GUSB (NP_000172.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:RTSH YPYA EEVM QMCD RYGI VVID ECPG VGLA LPQF FNNV SLHH HMQV MEEV VRRD KNHP AVVM WSVA NEPA SHLE SAGY YLKM VIAH TKSL DPSR PVTF VSNS NYAA DKGA PYVD VICL NSYY SWYH DYGH LELI QLQL ATQF ENWY KKYQ KPII QSEY GAET IAGF HQDP PLMF TEEY QKSL LEQY HLGL DQKR RKYV VGEL IWNF ADFM TEQS PTRV LGNK KGIF TRQR QPKS AAFL LRER YWKI ANET RYPH SVAK SQCL ENSL FT
Gene ID:2990
Uniprot:P08236
Cellular Location:Lysosome
Calculated MW:58kDa/69kDa/74kDa
Observed MW:75kDa
Synonyms:GUSB, BG, MPS7
Background: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.
UniProt Protein Function:GUSB: Plays an important role in the degradation of dermatan and keratan sulfates. 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. Belongs to the glycosyl hydrolase 2 family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Glycan Metabolism - glycosaminoglycan degradation; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Hydrolase; Carbohydrate Metabolism - starch and sucrose; Carbohydrate Metabolism - pentose and glucuronate interconversions; Xenobiotic Metabolism - drug metabolism - other enzymes; EC 3.2.1.31

Chromosomal Location of Human Ortholog: 7q21.11

Cellular Component: lysosomal lumen; membrane; intracellular membrane-bound organelle

Molecular Function:protein domain specific binding; beta-glucuronidase activity; receptor binding

Biological Process: glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis; hyaluronan metabolic process; hyaluronan catabolic process

Disease: Mucopolysaccharidosis, Type Vii

NCBI Summary: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]
UniProt Code:P08236
NCBI GenInfo Identifier:146345377
NCBI Gene ID:2990
NCBI Accession:P08236.2
UniProt Secondary Accession:P08236,Q549U0, Q96CL9, B4E1F6, E9PCV0,
UniProt Related Accession:P08236
Molecular Weight:651
NCBI Full Name:Beta-glucuronidase
NCBI Synonym Full Names:glucuronidase, beta
NCBI Official Symbol:GUSB  
NCBI Official Synonym Symbols:BG; MPS7  
NCBI Protein Information:beta-glucuronidase; beta-G1; beta-D-glucuronidase
UniProt Protein Name:Beta-glucuronidase
UniProt Synonym Protein Names:Beta-G1
Protein Family:Beta-glucuronidase
UniProt Gene Name:GUSB  
UniProt Entry Name:BGLR_HUMAN
Anti-GUSB Antibody (CAB5813)Western blot analysis of extracts of mouse skeletal muscle, using GUSB antibody (CAB5813) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s.
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