Testing Data
(Detection limit for recombinant GST tagged MPZ is ~1ng/ml as a capture antibody.)
Mouse anti-Human Myelin Protein Zero Monoclonal Antibody | anti-MPZ antibody
Myelin Protein Zero (Myelin Protein P0, MPZ, P0, CHM, CMT1, CMT1B, CMT2I, CMT2J, CMT4E, CMTDI3, DSS, HMSNIB, Myelin Peripheral Protein, MPP)
Purified by Protein A affinity chromatography.
Purified by Protein A affinity chromatography.
Testing Data
(Detection limit for recombinant GST tagged MPZ is ~1ng/ml as a capture antibody.)
Testing Data
(Detection limit for recombinant GST tagged MPZ is ~1ng/ml as a capture antibody.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a major structural protein of peripheral myelin. Mutations in this gene result in the autosomal dominant form of Charcot-Marie-Tooth disease type 1 and other polyneuropathies. [provided by RefSeq, Apr 2010]
Uniprot Description
Function: Creation of an extracellular membrane face which guides the wrapping process and ultimately compacts adjacent lamellae.
Subunit structure: Homodimer and homotetramer
Probable. Ref.15
Subcellular location: Cell membrane; Single-pass type I membrane protein Ref.11. Isoform L-MPZ: Myelin membrane; Single-pass type I membrane protein Ref.11.
Tissue specificity: Found only in peripheral nervous system Schwann cells.
Post-translational modification: N-glycosylated; contains sulfate-substituted glycan
By similarity.
Involvement in disease: Charcot-Marie-Tooth disease 1B (CMT1B) [MIM:118200]: A dominant demyelinating form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Demyelinating neuropathies 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.2 Ref.9 Ref.10 Ref.16 Ref.17 Ref.18 Ref.19 Ref.21 Ref.22 Ref.23 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.34 Ref.35 Ref.36 Ref.40 Ref.43 Ref.44 Ref.45 Ref.47 Ref.50 Ref.51 Ref.53 Ref.54 Ref.55 Ref.56 Ref.58 Ref.60 Ref.61 Ref.62 Ref.64 Ref.69 Ref.70 Ref.72Charcot-Marie-Tooth disease 2I (CMT2I) [MIM:607677]: A dominant axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.37 Ref.54 Ref.63 Ref.66Charcot-Marie-Tooth disease 2J (CMT2J) [MIM:607736]: A dominant axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. Charcot-Marie-Tooth disease type 2J is characterized by the association of axonal peripheral neuropathy with hearing loss and pupillary abnormalities such as Adie pupil.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.39 Ref.49 Ref.68 Ref.73Adie pupil (ADIEP) [MIM:103100]: A stationary, benign disorder characterized by tonic, sluggishly reacting pupil and hypoactive or absent tendon reflexes. Adie pupil is a characteristic of Charcot-Marie-Tooth disease type 2J.Note: The disease is caused by mutations affecting the gene represented in this entry.Charcot-Marie-Tooth disease, dominant, intermediate type, D (CMTDID) [MIM:607791]: A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. The dominant intermediate type D is characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec.Note: The disease may be caused by mutations affecting the gene represented in this entry. Ref.42Dejerine-Sottas syndrome (DSS) [MIM:145900]: A severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.20 Ref.24 Ref.28 Ref.30 Ref.32 Ref.33 Ref.34 Ref.36 Ref.50 Ref.52 Ref.54 Ref.60Neuropathy, congenital hypomyelinating or amyelinating (CHN) [MIM:605253]: A severe degenerating neuropathy that results from a congenital impairment in myelin formation. It is clinically characterized by early onset of hypotonia, areflexia, distal muscle weakness, and very slow nerve conduction velocities (as low as 3m/s). Some patients manifest nearly complete absence of spontaneous limb movements, respiratory distress at birth, and complete absence of myelin shown by electron microscopy of peripheral nerves. Inheritance can be autosomal dominant or recessive.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.67Roussy-Levy syndrome (ROULS) [MIM:180800]: Autosomal dominant disorder that resembles Charcot-Marie-Tooth disease type 1 in that it presents with foot deformity, weakness and atrophy of distal limb muscles, especially the peronei, and absent tendon reflexes. The phenotype differs, however, in that it includes static tremor of the upper limbs and gait ataxia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.38
Sequence similarities: Belongs to the myelin P0 protein family.Contains 1 Ig-like V-type (immunoglobulin-like) domain.
Sequence caution: The sequence AAH06491.1 differs from that shown. Reason: Erroneous initiation. The sequence AAP35411.1 differs from that shown. Reason: Erroneous initiation. The sequence BAA03540.1 differs from that shown. Reason: Erroneous initiation. The sequence BAG36330.1 differs from that shown. Reason: Erroneous initiation. The sequence CAH70270.1 differs from that shown. Reason: Erroneous initiation. The sequence EAW52606.1 differs from that shown. Reason: Erroneous initiation.
Research Articles on MPZ
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Product Notes
The MPZ mpz (Catalog #AAA647579) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Myelin Protein Zero (Myelin Protein P0, MPZ, P0, CHM, CMT1, CMT1B, CMT2I, CMT2J, CMT4E, CMTDI3, DSS, HMSNIB, Myelin Peripheral Protein, MPP) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Myelin Protein Zero can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA). Suitable for use in ELISA. Researchers should empirically determine the suitability of the MPZ mpz 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: MLRAPAPAPA MAPGAPSSSP SPILAVLLFS SLVLSPAQAI VVYTDREAHG AVGSRVTLHC SFWSSEWVSD DISFTWRYQP EGGRDAISIF HYAKGQPYID EVGTFKERIQ WVGDPRWKDG SIVIHNLDYS DNGTFTCDVK NPPDIVGKTS QVTLYVFEKV PTRYGVVLGA VIGGVLGVVL LLLLLFYVVR YCWLRRQAAL QRRLSAMEKG KLHKPGKDAS KRGRQTPVLY AMLDHSRSTK AVSEKKAKGL GESRKDKK. It is sometimes possible for the material contained within the vial of "Myelin Protein Zero, Monoclonal 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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