Western Blot (WB)
(WB Suggested Anti-PMP22 AntibodyTitration: 1.0 ug/mlPositive Control: 721_B Whole Cell)
Rabbit PMP22 Polyclonal Antibody | anti-PMP22 antibody
PMP22 Antibody - N-terminal region
Western Blot (WB)
(WB Suggested Anti-PMP22 AntibodyTitration: 1.0 ug/mlPositive Control: 721_B Whole Cell)
Western Blot (WB)
(WB Suggested Anti-PMP22 AntibodyTitration: 1.0 ug/mlPositive Control: 721_B Whole Cell)
Target Description: 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. Alternative splicing of this gene results in three transcript variants that encode the same protein.
NCBI and Uniprot Product Information
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
Research Articles on PMP22
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Product Notes
The PMP22 pmp22 (Catalog #AAA3215931) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PMP22 Antibody - N-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Rabbit, Rat, Sheep and may cross-react with other species as described in the data sheet. AAA Biotech's PMP22 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the PMP22 pmp22 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: QWIVGNGHAT DLWQNCSTSS SGNVHHCFSS SPNEWLQSVQ ATMILSIIFS. It is sometimes possible for the material contained within the vial of "PMP22, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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