Name: Anti-GBE1 Antibody (CAB6599)
Brand: Metabolism Antibodies 2
SKU: CAB6599
Availability: OutOfStock

Antibody Name:	Anti-GBE1 Antibody
Antibody SKU:	CAB6599
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse, Rat
Positive Samples:	HepG2, NCI-H460, Mouse heart, Mouse liver, Mouse kidney, Rat liver, Rat heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human GBE1 (NP_000149.3).
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:	MAAP MTPA ARPE DYEA ALNA ALAD VPEL ARLL EIDP YLKP YAVD FQRR YKQF SQIL KNIG ENEG GIDK FSRG YESF GVHR CADG GLYC KEWA PGAE GVFL TGDF NGWN PFSY PYKK LDYG KWEL YIPP KQNK SVLV PHGS KLKV VITS KSGE ILYR ISPW AKYV VREG DNVN YDWI HWDP EHSY EFKH SRPK KPRS LRIY ESHV GISS HEGK VASY KHFT CNVL PRIK GLGY NCIQ LMAI MEHA YYAS FGYQ ITSF FAAS SRYG TPEE LQEL VDTA HSMG IIVL LDVV HSHA SKNS ADGL
Gene ID:	2632
Uniprot:	Q04446
Cellular Location:
Calculated MW:	80kDa
Observed MW:	80kDa

Synonyms:

GBE1, APBD, GBE, GSD4, 1

Background:

The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1, 4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1, 6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease).

UniProt Protein Function:	GBE1: a monomeric enzyme that functions in glycogen symthesis by catalyzing the formation of alpha 1,6- glucosidic linkages.Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. It is most highly expressed in liver and muscle. Deficiency can result in glycogen storage disease IV (Andersen's disease).
UniProt Protein Details:	Protein type:EC 2.4.1.18; Carbohydrate Metabolism - starch and sucrose; Transferase Chromosomal Location of Human Ortholog: 3p12.3 Cellular Component: cytosol Molecular Function:cation binding; 1,4-alpha-glucan branching enzyme activity; hydrolase activity, hydrolyzing O-glycosyl compounds Biological Process: glycogen metabolic process; generation of precursor metabolites and energy; glycogen biosynthetic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis Disease: Polyglucosan Body Disease, Adult Form; Glycogen Storage Disease Iv
NCBI Summary:	The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease). [provided by RefSeq, Jul 2008]
UniProt Code:	Q04446
NCBI GenInfo Identifier:	357529509
NCBI Gene ID:	2632
NCBI Accession:	Q04446.3
UniProt Secondary Accession:	Q04446,Q96EN0, B3KWV3,
UniProt Related Accession:	Q04446
Molecular Weight:	702
NCBI Full Name:	1,4-alpha-glucan-branching enzyme
NCBI Synonym Full Names:	glucan (1,4-alpha-), branching enzyme 1
NCBI Official Symbol:	GBE1
NCBI Official Synonym Symbols:	GBE; APBD; GSD4
NCBI Protein Information:	1,4-alpha-glucan-branching enzyme; brancher enzyme; glycogen branching enzyme; glycogen-branching enzyme; amylo-(1,4 to 1,6) transglucosidase; amylo-(1,4 to 1,6) transglycosylase
UniProt Protein Name:	1,4-alpha-glucan-branching enzyme
UniProt Synonym Protein Names:	Brancher enzyme; Glycogen-branching enzyme
Protein Family:	1,4-alpha-glucan branching enzyme
UniProt Gene Name:	GBE1
UniProt Entry Name:	GLGB_HUMAN

Western blot analysis of extracts of various cell lines, using GBE1 antibody (CAB6599) 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 Basic Kit (RM00020). Exposure time: 90s.

Antibodies

GBE1 Antibody (PACO09424)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)