Anti-Peripheral Myelin Protein 22 (PMP22), clone CF1 (mouse monoclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
H	IHC	M	Purified	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

IHC

Purified

Monoclonal Antibody

Description
Catalogue Number	MAB2265
Description	Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1
Alternate Names	peripheral myelin protein 22 Growth arrest-specific protein 3 growth arrest-specific 3
Background Information	Peripheral myelin protein 22 (PMP22), a 160 amino acid glycoprotein, belongs to the claudin family of proteins. PMP22 is thought to have a critical role in external mesaxon formation during development as well as potential involvement in the progression of axon myelination. This glycoprotein has four hydrophobic domains a feature which gives rise to another potential role for PMP22 in the structure of peripheral nerve myelin. PMP22 has been observed in compact myelin found within the peripheral nerve of human adult, and has been noted as having a distribution similar to that of myelin Protein zero (PO). Defects in PMP-22 expression are causal to several hereditary demyelinating neuropathies including; inflammatory demyelinating polyneuropathy (IDP), hereditary neuropathy with liability to pressure palsies (HNPP), Charcot-Marie-Tooth disease Type 1A (CMT1A) and type 1E (CMT1E), and Dejerine-Sottas syndrome (DSS).

Description

Catalogue Number

MAB2265

Description

Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1

Alternate Names

peripheral myelin protein 22
Growth arrest-specific protein 3
growth arrest-specific 3

Background Information

Peripheral myelin protein 22 (PMP22), a 160 amino acid glycoprotein, belongs to the claudin family of proteins. PMP22 is thought to have a critical role in external mesaxon formation during development as well as potential involvement in the progression of axon myelination. This glycoprotein has four hydrophobic domains a feature which gives rise to another potential role for PMP22 in the structure of peripheral nerve myelin. PMP22 has been observed in compact myelin found within the peripheral nerve of human adult, and has been noted as having a distribution similar to that of myelin Protein zero (PO). Defects in PMP-22 expression are causal to several hereditary demyelinating neuropathies including; inflammatory demyelinating polyneuropathy (IDP), hereditary neuropathy with liability to pressure palsies (HNPP), Charcot-Marie-Tooth disease Type 1A (CMT1A) and type 1E (CMT1E), and Dejerine-Sottas syndrome (DSS).

Product Information
Format	Purified
Control	Human normal cortex tissue
Presentation	Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Product Information

Format

Purified

Control

Human normal cortex tissue

Presentation

Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Applications
Application	Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 is an antibody against Peripheral Myelin Protein 22 (PMP22) for use in IH.
Key Applications	Immunohistochemistry

Applications

Application

Anti-Peripheral Myelin Protein 22 (PMP22) Antibody, clone CF1 is an antibody against Peripheral Myelin Protein 22 (PMP22) for use in IH.

Key Applications

Immunohistochemistry

Biological Information
Immunogen	Human PMP22 cDNA boosted with 13-mer peptide of the second extracellular domain of PMP22
Epitope	Unknown
Clone	CF1
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	This antibody recognizes peripheral myelin protein 22 (PMP22)
Isotype	IgG1κ
Species Reactivity	Human
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NP_000295
Entrez Gene Summary	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. [provided by RefSeq].
Gene Symbol	PMP22 GAS3 CMT1A PMP-22 DSS CMT1E GAS-3 HMSNIA HNPP Sp110
Purification Method	Protein G
UniProt Number	Q01453
UniProt Summary	FUNCTION: Might be involved in growth regulation, and in myelinization in the peripheral nervous system. SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein. INVOLVEMENT IN DISEASE: Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; 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) [MIM:145900]; 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. Ref.10 Ref.11 Ref.14 Ref.15 Ref.18 Ref.19 Ref.20 Ref.21 Ref.24 Ref.25 Ref.26 Ref.28 Ref.31 Ref.33 Ref.37 Ref.41 Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP) [MIM:162500]; an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas. Ref.29 Ref.44 Ref.45 Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E) [MIM:118300]; 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) [MIM:139393]. 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. SEQUENCE SIMILARITIES: Belongs to the PMP-22/EMP/MP20 family.
Molecular Weight	18 kDa calculated

Biological Information

Immunogen

Human PMP22 cDNA boosted with 13-mer peptide of the second extracellular domain of PMP22

Epitope

Unknown

Clone

CF1

Concentration

Please refer to the Certificate of Analysis for the lot-specific concentration.

Host

Mouse

Specificity

This antibody recognizes peripheral myelin protein 22 (PMP22)

Isotype

IgG1κ

Species Reactivity

Human

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NP_000295

Entrez Gene Summary

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. [provided by RefSeq].

Gene Symbol

PMP22
GAS3
CMT1A
PMP-22
DSS
CMT1E
GAS-3
HMSNIA
HNPP
Sp110

Purification Method

Protein G

UniProt Number

Q01453

UniProt Summary

FUNCTION: Might be involved in growth regulation, and in myelinization in the peripheral nervous system.

SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein.

INVOLVEMENT IN DISEASE: Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; 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) [MIM:145900]; 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. Ref.10 Ref.11 Ref.14 Ref.15 Ref.18 Ref.19 Ref.20 Ref.21 Ref.24 Ref.25 Ref.26 Ref.28 Ref.31 Ref.33 Ref.37 Ref.41

Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP) [MIM:162500]; an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas. Ref.29 Ref.44 Ref.45

Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E) [MIM:118300]; 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) [MIM:139393]. 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.

SEQUENCE SIMILARITIES: Belongs to the PMP-22/EMP/MP20 family.

Molecular Weight

18 kDa calculated

Product Usage Statements
Quality Assurance	Evaluated by Immunohistochemistry in human normal cortex tissue. Immunohistochemistry Analysis: 1:500 dilution of this antibody detected PMP22 in human normal cortex tissue.
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Quality Assurance

Evaluated by Immunohistochemistry in human normal cortex tissue.

Immunohistochemistry Analysis: 1:500 dilution of this antibody detected PMP22 in human normal cortex tissue.

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Stable for 1 year at 2-8°C from date of receipt.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at 2-8°C from date of receipt.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Anti-Peripheral Myelin Protein 22 (PMP22), clone CF1 (mouse monoclonal)

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