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Recombinant Mouse Anti-PMP22 Antibody (CBHb267) (CBMAB-Hb866-LY)

The product is antibody recognizes PMP22. The antibody CBHb267 immunoassay techniques such as: Immuno assays, IHC-P, WB.
See all PMP22 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Monkey
Clone
CBHb267
Antibody Isotype
IgG1
Application
Immuno assays, IHC-P, WB

Basic Information

Immunogen
Peptide corresponding to amino acids 121-133 in the second extracellular domain of human PMP2 with an extra N-terminal cysteine residue (CRHPEWHLNSDYSYG)
Specificity
Human, Monkey
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
0.1% BSA
Preservative
0.02% sodium azide
Concentration
0.1 mg/ml
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
peripheral myelin protein 22
Entrez Gene ID
Human5376
Monkey693527
UniProt ID
HumanQ01453
MonkeyF6W2G0
Alternative Names
Growth arrest-specific protein 3, Gas-3
Function
Might be involved in growth regulation, and in myelinization in the peripheral nervous system.
Biological Process
Bleb assemblyManual Assertion Based On ExperimentIDA:UniProtKB
Cell deathManual Assertion Based On ExperimentIDA:UniProtKB
Cell differentiationIEA:Ensembl
Chemical synaptic transmissionManual Assertion Based On ExperimentTAS:ProtInc
Myelin assemblyManual Assertion Based On ExperimentIBA:GO_Central
Negative regulation of cell population proliferationIEA:Ensembl
Negative regulation of neuron projection developmentIEA:Ensembl
Peripheral nervous system developmentManual Assertion Based On ExperimentTAS:ProtInc
Cellular Location
Cell membrane
Involvement in disease
Charcot-Marie-Tooth disease 1A (CMT1A):
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.
Dejerine-Sottas syndrome (DSS):
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.
Hereditary neuropathy with liability to pressure palsies (HNPP):
A neurologic disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas.
Charcot-Marie-Tooth disease 1E (CMT1E):
An autosomal dominant form of Charcot-Marie-Tooth disease characterized by the association of sensorineural hearing loss with peripheral demyelinating neuropathy.
Inflammatory demyelinating polyneuropathy (IDP):
Putative autoimmune disorder presenting in an acute (AIDP) or chronic form (CIDP). The acute form is also known as Guillain-Barre syndrome.
Topology
Cytoplasmic: 1
Helical: 2-31
Extracellular: 32-64
Helical: 65-91
Cytoplasmic: 92-95
Helical: 96-119
Extracellular: 120-133
Helical: 134-156
Cytoplasmic: 157-160
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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