EGR2, Blocking Peptide

For Research Use Only. Not for use in diagnostic procedures.

Lyophilized powder

Add 100ul of sterile PBS. Final peptide concentration is 1 mg/ml in PBS. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.

Small volumes of EGR2 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This is a synthetic peptide designed for use in combination with anti-EGR2 Antibody, made

Target Description: The protein encoded by this gene is a transcription factor with three tandem C2H2-type zinc fingers. Defects in this gene are associated with Charcot-Marie-Tooth disease type 1D (CMT1D), Charcot-Marie-Tooth disease type 4E (CMT4E), and with Dejerine-Sottas syndrome (DSS). Multiple transcript variants encoding two different isoforms have been found for this gene.

Peptide

Western Blot (WB)

46kDa

early growth response 2

E3 SUMO-protein ligase EGR2

E3 SUMO-protein ligase EGR2

KROX20; EGR-2  [Similar Products]

EGR2_HUMAN

The protein encoded by this gene is a transcription factor with three tandem C2H2-type zinc fingers. Defects in this gene are associated with Charcot-Marie-Tooth disease type 1D (CMT1D), Charcot-Marie-Tooth disease type 4E (CMT4E), and with Dejerine-Sottas syndrome (DSS). Multiple transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]

EGR2: Sequence-specific DNA-binding transcription factor. Binds to two specific DNA sites located in the promoter region of HOXA4. Defects in EGR2 are a cause of congenital hypomyelination neuropathy (CHN). Inheritance can be autosomal dominant or recessive. Recessive CHN is also known as Charcot- Marie-Tooth disease type 4E (CMT4E). CHN is characterized clinically by early onset of hypotonia, areflexia, distal muscle weakness, and very slow nerve conduction velocities. Defects in EGR2 are a cause of Charcot-Marie-Tooth disease type 1D (CMT1D). CMT1D is a form of Charcot- Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. Defects in EGR2 are a cause of Dejerine-Sottas syndrome (DSS); also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie- Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome. Belongs to the EGR C2H2-type zinc-finger protein family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: DNA-binding; C2H2-type zinc finger protein; EC 6.3.2.-

Chromosomal Location of Human Ortholog: 10q21.1

Cellular Component: cytoplasm; nucleus

Molecular Function: protein binding; ubiquitin protein ligase binding; metal ion binding; chromatin binding; transcription factor activity; ligase activity

Biological Process: myelination; transcription from RNA polymerase II promoter; fat cell differentiation; facial nerve structural organization; rhombomere 5 formation; positive regulation of transcription, DNA-dependent; motor axon guidance; response to insulin stimulus; peripheral nervous system development; Schwann cell differentiation; rhythmic behavior; protein sumoylation; learning and/or memory; rhombomere 3 formation; positive regulation of transcription from RNA polymerase II promoter; brain development; protein export from nucleus; regulation of ossification; brain segmentation; regulation of neuronal synaptic plasticity; negative regulation of apoptosis

Disease: Neuropathy, Congenital Hypomyelinating Or Amyelinating, Autosomal Recessive; Hypertrophic Neuropathy Of Dejerine-sottas; Charcot-marie-tooth Disease, Demyelinating, Type 1d

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