-
Type
Conjugated Primary Antibody
-
Conjugated with
ALEXA FLUOR® 594
-
Host organism
Rabbit (Oryctolagus cuniculus)
-
Target Protein Peptide
GBE1
-
Specificity
This antibody reacts specifically with GBE1
-
Modification
No modification has been applied to this antibody
-
Modification site
None
-
Clonality
Polyclonal Antibody
-
Clone
Polyclonal Antibodies
-
Concentration
1ug per 1ul
-
Subcellular locations
N/A
-
Antigen Source
KLH conjugated synthetic peptide derived from human GBE1
-
Gene ID
2632
-
Swiss Prot
N/A
-
Applications
IF(IHC-P)
-
Applications with corresponding dilutions
IF(IHC-P)(1:50-200)
-
Cross reactive species
Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)
-
Cross Reactive Species details
No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.
-
Background information
GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 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 a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.
-
Purification method
Purified by Protein A.
-
Storage
Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
-
Excitation emission
590nm/617nm
-
Synonyms
1,4 alpha glucan branching enzyme; 4-alpha-glucan-branching enzyme; amylo 1,4 to 1,6 transglucosidase; amylo 1,4 to 1,6 transglycosylase; Andersen disease; Brancher enzyme; GBE 1; GBE; GBE1; gGlucan 1,4 alpha , branching enzyme 1; GLGB_HUMAN; Glucan 1,4 alpha branching enzyme; Glycogen branching enzyme; Glycogen storage disease type IV; Glycogen-branching enzyme; OTTHUMP00000213788; OTTHUMP00000213833.
-
Also known as
GBE1 Polyclonal Antibody
-
Other name
Anti-GBE1 Polyclonal
-
Advisory
Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.
-
Properties
For facs or microscopy Alexa 1 conjugate.
-
Conjugation
Alexa Fluor, ALEXA FLUOR® 594
-
Conjugated
Alexa conjugate 1
-
Description
This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided. Antibody for research use.
-
Group
Polyclonals and antibodies
-
About
Polyclonals can be used for Western blot, immunohistochemistry on frozen slices or parrafin fixed tissues. The advantage is that there are more epitopes available in a polyclonal antiserum to detect the proteins than in monoclonal sera.
