Product datasheet
Anti-GJC2 antibody ab176300
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Overview
Product name
Anti-GJC2 antibody
Description
Rabbit polyclonal to GJC2
Tested applications
WB
Species reactivity
Reacts with: Mouse
Predicted to work with: Rat, Human
Immunogen
Recombinant full length protein corresponding to Human GJC2 aa 1-493.
Sequence:
MTNMSWSFLT RLLEEIHNHS TFVGKVWLTV
LVVFRIVLTA VGGEAIYSDE QAKFTCNTRQ
PGCDNVCYDA FAPLSHVRFW VFQIVVISTP
SVMYLGYAVH RLARASEQER RRALRRRPGP
RRAPRAHLPP PHAGWPEPAD LGEEEPMLGL
GEEEEEEETG AAEGAGEEAE EAGAEEACTK
AVGADGKAAG TPGPTGQHDG RRRIQREGLM
RVYVAQLVAR AAFEVAFLVG QYLLYGFEVR
PFFPCSRQPC PHVVDCFVSR PTEKTVFLLV
MYVVSCLCLL LNLCEMAHLG LGSAQDAVRG
RRGPPASAPA PAPRPPPCAF PAAAAGLACP
PDYSLVVRAA ERARAHDQNL ANLALQALRD
GAAAGDRDRD SSPCVGLPAA SRGPPRAGAP
ASRTGSATSA GTVGEQGRPG THERPGAKPR
AGSEKGSASS RDGKTTVWI
Database link: Q5T442
Run BLAST with
Positive control
Run BLAST with
Mouse fetal muscle and OP9 lysates.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long
term. Avoid freeze / thaw cycle.
Storage buffer
pH: 7.3
Preservative: 0.02% Sodium azide
Constituents: 49% PBS, 50% Glycerol
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Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Applications
Our Abpromise guarantee covers the use of ab176300 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application
WB
Abreviews
Notes
1/500 - 1/2000. Predicted molecular weight: 47 kDa.
Target
Function
One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the
connexons, through which materials of low MW diffuse from one cell to a neighboring cell. May
play a role in myelination in central and peripheral nervous systems.
Tissue specificity
Expressed in central nervous system, in sciatic nerve and sural nerve. Also detected in skeletal
muscles.
Involvement in disease
Defects in GJC2 are the cause of leukodystrophy hypomyelinating type 2 (HLD2) [MIM:608804];
also known as Pelizaeus-Merzbacher-like disease autosomal recessive type 1. HLD2 is an
autosomal recessive hypomyelinating leukodystrophy characterized by nystagmus, impaired
motor development, ataxia, choreoathetotic movements, dysarthria and progressive spasticity.
Defects in GJC2 are the cause of spastic paraplegia autosomal recessive type 44 (SPG44)
[MIM:613206]. A form of spastic paraplegia, a neurodegenerative disorder characterized by a
slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and
the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance,
weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In
some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the
weakness and stiffness may spread to other parts of the body.
Defects in GJC2 are the cause of lymphedema hereditary type 1C (LMPH1C) [MIM:613480].
LMPH1C is a chronic disabling condition which results in swelling of the extremities due to
altered lymphatic flow. Patients with lymphedema suffer from recurrent local infections and
physical impairment.
Sequence similarities
Belongs to the connexin family. Gamma-type subfamily.
Cellular localization
Cell membrane. Cell junction > gap junction.
Anti-GJC2 antibody images
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All lanes : Anti-GJC2 antibody (ab176300)
at 1/500 dilution
Lane 1 : Mouse fetal lysate
Lane 2 : OP9 cell lysate
Western blot - Anti-GJC2 antibody (ab176300)
Predicted band size : 47 kDa
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