Product details

Subcellular locations

N/A

Swiss Prot

N/A

Gene ID

2632

Target Protein/Peptide

GBE1

Modification site

None

Applications

IF(IHC-P)

Excitation emission

590nm/617nm

Concentration

1ug per 1ul

Conjugated

Alexa conjugate 1

Conjugated with

ALEXA FLUOR® 594

Applications with corresponding dilutions

IF(IHC-P)(1:50-200)

Clonality

Polyclonal Antibody

Other name

Anti-GBE1 Polyclonal

Clone

Polyclonal Antibodies

Purification method

Purified by Protein A.

Also known as

GBE1 Polyclonal Antibody

Group

Polyclonals and antibodies

Type

Conjugated Primary Antibody

Conjugation

Alexa Fluor,ALEXA FLUOR® 594

Host organism

Rabbit (Oryctolagus cuniculus)

Properties

For facs or microscopy Alexa 1 conjugate.

Specificity

This antibody reacts specifically with GBE1

Modification

No modification has been applied to this antibody

Antigen Source

KLH conjugated synthetic peptide derived from human GBE1

Cross reactive species

Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)

Storage

Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.

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.

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.

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.

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.

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.

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.

Anti-GBE1 (Polyclonal), ALEXA Fluor 594

Description:

Size: 100 microliters

Catalog no.: GENTObs-13300R-A594

Price: 489 EUR