Human Myelin Protein P0 (MPZ) ELISA Kit | MPZ elisa kit
Human Myelin Protein P0 (MPZ) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Background: Myelin protein zero contains a large hydrophobic extracellular domain and a smaller basic intracellular domain, which are essential for the formation and stabilization of the multilamellar structure of the compact myelin. Mutations in MPZ are associated with autosomal dominant form of Charcot-Marie-Tooth disease type 1 (CMT1B) and other polyneuropathies, such as Dejerine-Sottas syndrome (DSS) and congenital hypomyelinating neuropathy (CHN). A recent study showed that two isoforms are produced from the same mRNA by use of alternative in-frame translation termination codons via a stop codon readthrough mechanism.
NCBI and Uniprot Product Information
NCBI Description
This gene is specifically expressed in Schwann cells of the peripheral nervous system and encodes a type I transmembrane glycoprotein that is a major structural protein of the peripheral myelin sheath. The encoded protein contains a large hydrophobic extracellular domain and a smaller basic intracellular domain, which are essential for the formation and stabilization of the multilamellar structure of the compact myelin. Mutations in this gene are associated with autosomal dominant form of Charcot-Marie-Tooth disease type 1 (CMT1B) and other polyneuropathies, such as Dejerine-Sottas syndrome (DSS) and congenital hypomyelinating neuropathy (CHN). A recent study showed that two isoforms are produced from the same mRNA by use of alternative in-frame translation termination codons via a stop codon readthrough mechanism. [provided by RefSeq, Oct 2015]
Uniprot Description
myelin P0: a structural protein in peripheral nervous system Schwann cells. Forms an extracellular membrane face which guides the wrapping process and ultimately compacts adjacent lamellae. Defects cause of Charcot-Marie-Tooth disease, Dejerine-Sottas syndrome, congenital hypomyelination neuropathy, and Roussy-Levy syndrome.
Protein type: Adaptor/scaffold; Membrane protein, integral
Chromosomal Location of Human Ortholog: 1q23.3
Cellular Component: integral to plasma membrane; plasma membrane; myelin sheath
Molecular Function: structural molecule activity
Biological Process: synaptic transmission; intercellular junction maintenance
Disease: Neuropathy, Congenital Hypomyelinating Or Amyelinating, Autosomal Recessive; Charcot-marie-tooth Disease, Demyelinating, Type 1b; Hypertrophic Neuropathy Of Dejerine-sottas; Charcot-marie-tooth Disease, Dominant Intermediate D; Charcot-marie-tooth Disease, Axonal, Type 2j; Charcot-marie-tooth Disease, Axonal, Type 2i; Roussy-levy Hereditary Areflexic Dystasia
Research Articles on MPZ
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Product Notes
The Human MPZ mpz (Catalog #AAA9715212) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9715212 ELISA Kit recognizes Human MPZ. It is sometimes possible for the material contained within the vial of "Myelin Protein P0 (MPZ), ELISA Kit" 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
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