Rabbit anti-Human PMP22 Polyclonal Antibody | anti-PMP22 antibody

PMP22 (PMP22, GAS3, Peripheral myelin protein 22, Growth arrest-specific protein 3) (FITC)

Cat. Num. AAA6335183

• Gene Names: PMP22; DSS; CIDP; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: PMP22; Polyclonal Antibody; PMP22 (PMP22; GAS3; Peripheral myelin protein 22; Growth arrest-specific protein 3) (FITC); Growth arrest-specific protein 3; anti-PMP22 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
• Sequence Length: 160

• Applicable Applications for anti-PMP22 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: PMP22 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from 110-140 human PMP22.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(PMP22 Antibody western blot analysis in human normal Uterus tissue lysates (35ug/lane).This demonstrates the PMP22 antibody detected the PMP22 protein (arrow).)

Related Product Information for anti-PMP22 antibody

This gene encodes an integral membrane protein that is a major component of myelin in the peripheral nervous system. Various mutations of this gene are causes of Charcot-Marie-Tooth disease Type IA, Dejerine-Sottas syndrome, and hereditary neuropathy with liability to pressure palsies.

Product Categories/Family for anti-PMP22 antibody

Antibodies; Myelin

References

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010). Kohl, B., et al. Am. J. Pathol. 176(3):1390-1399(2010). Kabzinska, D., et al. J. Appl. Genet. 51(2):203-209(2010). Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009). Stangler Herodez, S., et al. J. Int. Med. Res. 37(5):1626-1631(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 24430163
• NCBI GeneID: 5376
• NCBI Accession #: NP_696996.1; NP_000295.1; NP_696997.1
• NCBI GenBank Nucleotide #: NP_696996.1
• UniProt Accession #: Q01453
• NCBI Official Full Name: peripheral myelin protein 22 isoform 1
• NCBI Official Synonym Full Names: peripheral myelin protein 22
• NCBI Official Symbol: PMP22
• NCBI Official Synonym Symbols: DSS; CIDP; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• NCBI Protein Information: peripheral myelin protein 22
• UniProt Protein Name: Peripheral myelin protein 22
• Protein Family: Peripheral myelin protein
• UniProt Gene Name: PMP22
• UniProt Synonym Gene Names: GAS3; PMP-22; GAS-3
• UniProt Entry Name: PMP22_HUMAN

NCBI Description

This gene encodes an integral membrane protein that is a major component of myelin in the peripheral nervous system. Studies suggest two alternately used promoters drive tissue-specific expression. Various mutations of this gene are causes of Charcot-Marie-Tooth disease Type IA, Dejerine-Sottas syndrome, and hereditary neuropathy with liability to pressure palsies. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

Uniprot Description

PMP22: Might be involved in growth regulation, and in myelinization in the peripheral nervous system. Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A); also known as hereditary motor and sensory neuropathy IA. CMT1A 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. CMT1A inheritance is autosomal dominant. Defects in PMP22 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. Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP); an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas. Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E); also known as Charcot-Marie- Tooth disease and deafness autosomal dominant. CMT1E is an autosomal dominant form of Charcot-Marie-Tooth disease characterized by the association of sensorineural hearing loss with peripheral demyelinating neuropathy. Defects in PMP22 may be a cause of inflammatory demyelinating polyneuropathy (IDP). IDP is a putative autoimmune disorder presenting in an acute (AIDP) or chronic form (CIDP). The acute form is also known as Guillain-Barre syndrome. Belongs to the PMP-22/EMP/MP20 family.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Cell cycle regulation; Cell adhesion

Chromosomal Location of Human Ortholog: 17p12

Cellular Component: compact myelin; tight junction; integral to membrane; plasma membrane

Molecular Function: protein binding

Biological Process: cell death; negative regulation of cell proliferation; myelin formation; synaptic transmission; bleb formation; peripheral nervous system development

Disease: Charcot-marie-tooth Disease, Demyelinating, Type 1a; Neuropathy, Hereditary, With Liability To Pressure Palsies; Hypertrophic Neuropathy Of Dejerine-sottas; Charcot-marie-tooth Disease And Deafness; Roussy-levy Hereditary Areflexic Dystasia; Guillain-barre Syndrome, Familial