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Rabbit Anti-GBE1 Antibody (CSB-PA754361ESR2HU)
Supplier: CUSABIO BIOTECH CO.
1 Antibody,4-alpha-glucan-branching enzyme Antibody,Brancher enzyme Antibody,Glycogen-branching enzyme Antibody,GBE1 AntibodyMore alternative names for the antibody
1 antibody|1,4 alpha glucan branching enzyme antibody|4-alpha-glucan-branching enzyme antibody|amylo (1,4 to 1,6) transglucosidase antibody|amylo (1,4 to 1,6) transglycosylase antibody|Andersen disease antibody|APBD antibody|Brancher enzyme antibody|GBE 1 antibody|GBE antibody|GBE1 antibody|gGlucan (1,4 alpha ), branching enzyme 1 antibody|GLGB_HUMAN antibody|Glucan (1,4 alpha) branching enzyme antibody|Glycogen branching enzyme antibody|Glycogen storage disease type IV antibody|Glycogen-branching enzyme antibody|GSD4 antibody|OTTHUMP00000213788 antibody|OTTHUMP00000213833 antibody
Anti-GBE1 antibody [EP11113] (ab180596)
Anti-GBE1 antibody [EP11113] (ab180596)
Recommended applications: ELISA, WB, IHC
Recommended dilution: User optimized
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human 1,4-alpha-glucan-branching enzyme protein (1-300AA)
ELISA, WB, IHC
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:493962( Cat), Entrez Gene:100034152( Horse), Entrez Gene:2632( Human), Entrez Gene:74185( Mouse), Entrez Gene:288333( Rat), Omim:607839( Human), SwissProt:Q6T308( Cat), SwissProt:Q6EAS5( Horse), SwissProt:Q04446( Human), SwissProt:Q9D6Y9( Mouse), Unigene:436062( Human), Unigene:396102( Mouse), Unigene:29938( Rat)
|Protein function|| |
Required for normal glycogen accumulation (PubMed:8463281, PubMed:26199317, PubMed:8613547). The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule (Probable). .
|Involvement in disease|| |
Glycogen storage disease 4 (GSD4) [MIM:232500]: A metabolic disorder characterized by the accumulation of an amylopectin-like polysaccharide. The typical clinical manifestation is liver disease of childhood, progressing to lethal hepatic cirrhosis. Most children with this condition die before two years of age. However, the liver disease is not always progressive. No treatment apart from liver transplantation has been found to prevent progression of the disease. There is also a neuromuscular form of glycogen storage disease type 4 that varies in onset (perinatal, congenital, juvenile, or adult) and severity. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Note=Neuromuscular perinatal glycogen storage disease type 4 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. .; Polyglucosan body neuropathy, adult form (APBN) [MIM:263570]: A late-onset, slowly progressive disorder affecting the central and peripheral nervous systems. Patients typically present after age 40 years with a variable combination of cognitive impairment, pyramidal tetraparesis, peripheral neuropathy, and neurogenic bladder. Other manifestations include cerebellar dysfunction and extrapyramidal signs. The pathologic hallmark of APBN is the widespread accumulation of round, intracellular polyglucosan bodies throughout the nervous system, which are confined to neuronal and astrocytic processes. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the glycosyl hydrolase 13 family. GlgB subfamily. Binds its carbohydrate substrate close to the active site, but also via regions close to the N-terminus; this may result in increased affinity and therefore increased catalytic efficiency.
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CUSABIO BIOTECH CO.
|Product type|| |
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