Recombinant Mouse Peripheral myelin protein 22 (Pmp22)

Code CSB-CF018241MO
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Source in vitro E.coli expression system
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Product Details

Target Names
Pmp22
Uniprot No.
Alternative Names
Pmp22; Gas-3; Gas3; Pmp-22; Peripheral myelin protein 22; PMP-22; Growth arrest-specific protein 3; GAS-3
Species
Mus musculus (Mouse)
Expression Region
1-161
Target Protein Sequence
MLLLLLGILFLHIAVLVLLFVSTIVSQWLVGNGHTTDLWQNCTTSALGAVQHCYSSSVSE WLQSVQATMILSVIFSVLALFLFFCQLFTLTKGGRFYITGFFQILAGLCVMSAAAIYTVR HSEWHVNTDYSYGFAYILAWVAFPLALLSGIIYVILRKREL
Protein Length
Full length protein
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Function
Might be involved in growth regulation, and in myelinization in the peripheral nervous system.
Gene References into Functions
  1. selective suppression of the Pmp22 mutant allele by non-viral delivery of siRNA alleviates the demyelinating neuropathic phenotypes of Charcot-Marie-Tooth disease in vivo PMID: 28108290
  2. We discovered that Tead1 and co-activators Yap and Taz are required for Pmp22 expression, as well as for the expression of Egr2 Tead1 directly binds Pmp22 and Egr2 enhancers early in development and Tead1 binding is induced during myelination, correlating with Pmp22 expression. The data identify Tead1 as a novel regulator of Pmp22 expression during development in concert with Sox10 and Egr2 PMID: 27288457
  3. The basal lamina and PMP22 act in concert to contribute to a resilience and integrity of peripheral nerves at the single fibre level. PMID: 25446378
  4. A role was identified for PMP22 in the linkage of the actin cytoskeleton with the plasma membrane. PMID: 25429154
  5. This study demonistrated that Paranodal dysmyelination in peripheral nerves of Trembler mice. PMID: 24446165
  6. This study showed that a number of ongoing pathogenic mechanisms contribute to the progression of the neuropathy in C22 mice, which initiates with abnormal expression of PMP22. PMID: 24175617
  7. This study revealed a novel mechanism by which PMP22 deficiency affects nerve conduction not through removal of myelin, but through disruption of myelin junctions PMID: 24339129
  8. This study showed that mouse PMP22 is palmitoylated at C85 and mutating C85S abolishes PMP22 palmitoylation. PMID: 23127255
  9. Peripheral myelin protein 22 (PMP22) performs distinct actions on the formation, maturation, degeneration and regeneration of sciatic nerve myelin sheath. PMID: 21824506
  10. Egr2 and Sox10 activity are directly involved in mediating the developmental induction of Pmp22 expression through an intronic enhancer. PMID: 21411665
  11. The results of this study demonstrated that a function of Pmp22 is to protect the nerve from mechanical injury. PMID: 20071523
  12. Part of the PMP22 gene contains the necessary information to mirror the endogenous expression pattern in peripheral nerves during development and regeneration and in mouse models of demyelination due to genetic lesions. PMID: 12056842
  13. Association of calnexin ex vivo: a basis for "gain-of-function" ER diseases. PMID: 12119418
  14. Aggresome formation has now been observed with two mutant PMP22s, the Tr- and TrJ-PMP22 when the proteasome is inhibited. two pathways of PMP22 degradation are present. PMID: 12127149
  15. Mutant pmp22 from less severely affected mutants occurs in large aggregates, while that from more severely affected mutants occurs in a diffuse perinuclear pattern. Pmp22 aggregates may be protective in this form of peripheral neuropathy. PMID: 12359155
  16. Multiple distinct signaling pathways regulating Pmp22 expression in myelination as well as in neurons converge on distinct segments of the PMP22 promoter region. PMID: 14664827
  17. Recessive mutations were uniquely distinguished from dominant mutations by both the low potential for aggregation and their trafficking to the cell surface. PMID: 15474367
  18. Increased expression of genes involved in cell cycle regulation and DNA replication is characteristic and specific for early development in Pmp22-deficient mice, supporting a primary function of PMP22 in the regulation of Schwann cell proliferation. PMID: 15755691
  19. PMP22 is a binding partner in the integrin alpha6beta4/laminin complex and is involved in mediating the interaction of Schwann cells with the extracellular environment. PMID: 16436605
  20. The beneficial effects of autophagy and chaperones in preventing the accumulation of misfolded PMP22 are additive and provide a potential avenue for therapeutic approaches in hereditary neuropathies linked to PMP22 mutations. PMID: 17174099
  21. Data show that Med25 is coordinately expressed with Pmp22 gene dosage and expression in transgenic mice and rats, and suggest a potential role of this protein in the molecular etiology of Charcot-Marie-Tooth disease. PMID: 19290556

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Involvement in disease
A defect in Pmp-22 is the cause of trembler (tr) phenotype. Trembler mice show a Schwann cells defect characterized by severe hypomyelination and continuing Schwann cells proliferation throughout life.
Subcellular Location
Cell membrane; Multi-pass membrane protein.
Protein Families
PMP-22/EMP/MP20 family
Tissue Specificity
Schwann cells of the peripheral nervous system.
Database Links
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