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Western Blot (WB) (Anti- GJC2 Picoband antibody, MBS178173, Western blottingAll lanes: Anti GJC2 (MBS178173) at 0.5ug/mlLane 1: Rat Brain Tissue Lysate at 50ugLane 2: Mouse Brain Tissue Lysate at 50ugLane 3: U20S Whole Cell Lysate at 40ugLane 4: HELA Whole Cell Lysate at 40ugPredicted bind size: 47KDObserved bind size: 47KD )

GJC2 Polyclonal Antibody | anti-GJC2 antibody

Anti-GJC2 Antibody

Gene Names
GJC2; Cx47; HLD2; GJA12; SPG44; CX46.6; LMPH1C; PMLDAR
Reactivity
Human, Mouse, Rat
Applications
Western Blot
Purity
Immunogen Affinity Purified
Synonyms
GJC2; Polyclonal Antibody; Anti-GJC2 Antibody; Gap junction gamma-2 protein; B230382L12Rik; Connexin 46.6; Connexin 47; Connexin-46.6; Connexin-47; Connexin46.6; Connexin47; CX 46.6; Cx 47; Cx46.6; Cx47; CXG2_HUMAN; Gap junction alpha 12 protein; Gap junction alpha-12 protein; Gap junction gamma 2 protein; Gap junction protein alpha 12 47kDa; Gap junction protein gamma 2 47kDa; GAP JUNCTION PROTEIN; 47-KD; gap junction protein; gamma 12; 47kDa; gamma 2; GJA 12; GJA12; GJC 2; Gjc2; HLD 2; HLD2; LMPH1C; MGC105119; MGC130506; OTTHUMP00000037763; PMLDAR; SPG44; anti-GJC2 antibody
Ordering
For Research Use Only!
Reactivity
Human, Mouse, Rat
Clonality
Polyclonal
Purity/Purification
Immunogen Affinity Purified
Form/Format
Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
Sequence Length
439
Applicable Applications for anti-GJC2 antibody
Western Blot (WB)
Application Notes
Western Blot Concentration: 0.1-0.5ug/ml
Immunogen
A synthetic peptide corresponding to a sequence at the C-terminus of human GJC2 (336-368aa VVRAAERARAHDQNLANLALQALRDGAAAGDRD), different from the related mouse and rat sequences by five amino acids.
Ig Type
Rabbit IgG
Reconstitution
Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Preparation and Storage
At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquoted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

(Anti- GJC2 Picoband antibody, MBS178173, Western blottingAll lanes: Anti GJC2 (MBS178173) at 0.5ug/mlLane 1: Rat Brain Tissue Lysate at 50ugLane 2: Mouse Brain Tissue Lysate at 50ugLane 3: U20S Whole Cell Lysate at 40ugLane 4: HELA Whole Cell Lysate at 40ugPredicted bind size: 47KDObserved bind size: 47KD )

Western Blot (WB) (Anti- GJC2 Picoband antibody, MBS178173, Western blottingAll lanes: Anti GJC2 (MBS178173) at 0.5ug/mlLane 1: Rat Brain Tissue Lysate at 50ugLane 2: Mouse Brain Tissue Lysate at 50ugLane 3: U20S Whole Cell Lysate at 40ugLane 4: HELA Whole Cell Lysate at 40ugPredicted bind size: 47KDObserved bind size: 47KD )
Related Product Information for anti-GJC2 antibody
Description: Rabbit IgG polyclonal antibody for Gap junction gamma-2 protein(GJC2) detection. Tested with WB in Human;Mouse;Rat.

Background: Gap junction gamma-2 (GJC2), also known as connexin-46.6 (Cx46.6) and connexin-47 (Cx47) and gap junction alpha-12 (GJA12), is a protein that in humans is encoded by the GJC2 gene. It is mapped to 1q42.13. This gene encodes a gap junction protein. Gap junction proteins are members of a large family of homologous connexins and comprise 4 transmembrane, 2 extracellular, and 3 cytoplasmic domains. This gene plays a key role in central myelination and is involved in peripheral myelination in humans.
References
1. Ferrell RE, Baty CJ, Kimak MA; et al. (2010). "GJC2 missense mutations cause human lymphedema.". Am. J. Hum. Genet. 86 (6): 943-8. 2. Ostergaard P, Simpson MA, Brice G; et al. (2011). "Rapid identification of mutations in GJC2 in primary lymphoedema using whole exome sequencing combined with linkage analysis with delineation of the phenotype.". J. Med. Genet. 48 (4): 251-5. 3. Wolf NI, Cundall M, Rutland P; et al. (2007). "Frameshift mutation in GJA12 leading to nystagmus, spastic ataxia and CNS dys-/demyelination.".Neurogenetics 8 (1): 39-44.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
47,002 Da
NCBI Official Full Name
gap junction gamma-2 protein
NCBI Official Synonym Full Names
gap junction protein gamma 2
NCBI Official Symbol
GJC2
NCBI Official Synonym Symbols
Cx47; HLD2; GJA12; SPG44; CX46.6; LMPH1C; PMLDAR
NCBI Protein Information
gap junction gamma-2 protein
UniProt Protein Name
Gap junction gamma-2 protein
UniProt Gene Name
GJC2
UniProt Synonym Gene Names
GJA12; Cx46.6; Cx47
UniProt Entry Name
CXG2_HUMAN

NCBI Description

This gene encodes a gap junction protein. Gap junction proteins are members of a large family of homologous connexins and comprise 4 transmembrane, 2 extracellular, and 3 cytoplasmic domains. This gene plays a key role in central myelination and is involved in peripheral myelination in humans. Defects in this gene are the cause of autosomal recessive Pelizaeus-Merzbacher-like disease-1. [provided by RefSeq, Jul 2008]

Uniprot Description

GJA12: 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. Defects in GJC2 are the cause of leukodystrophy hypomyelinating type 2 (HLD2); 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). 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). 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. Belongs to the connexin family. Gamma-type subfamily.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 1q42.13

Cellular Component: connexon complex; integral to membrane; myelin sheath

Molecular Function: gap junction channel activity

Biological Process: cell-cell signaling; response to toxin; transmembrane transport

Disease: Leukodystrophy, Hypomyelinating, 2; Lymphedema, Hereditary, Ic; Spastic Paraplegia 44, Autosomal Recessive

Research Articles on GJC2

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Product Notes

The GJC2 gjc2 (Catalog #AAA178173) is an Antibody and is intended for research purposes only. The product is available for immediate purchase. The Anti-GJC2 Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GJC2 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western Blot Concentration: 0.1-0.5ug/ml. Researchers should empirically determine the suitability of the GJC2 gjc2 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. It is sometimes possible for the material contained within the vial of "GJC2, Polyclonal 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.

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