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Mouse Anti-GAN Recombinant Antibody (4G7) (CBMAB-G1885-LY)

This product is antibody recognizes GAN. The antibody 4G7 immunoassay techniques such as: ELISA, IF, WB.
See all GAN antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4G7
Antibody Isotype
IgG2a, κ
Application
ELISA, IF, WB

Basic Information

Immunogen
GAN (NP_071324, 534 a.a. ~ 597 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: DLDTGTNYDY VREFKRSTGT WHHTKPLLPS DLRRTGCAAL RIANCKLFRL QLQQGLFRIR VHSP
Specificity
Human
Antibody Isotype
IgG2a, κ
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
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
gigaxonin
Introduction
This gene encodes a member of the cytoskeletal BTB/kelch (Broad-Complex, Tramtrack and Bric a brac) repeat family. The encoded protein plays a role in neurofilament architecture and is involved in mediating the ubiquitination and degradation of some proteins. Defects in this gene are a cause of giant axonal neuropathy (GAN). [provided by RefSeq, Oct 2008]
Entrez Gene ID
UniProt ID
Alternative Names
Gigaxonin; Kelch-Like Family Member 16; Kelch-Like Protein 16; KLHL16; GAN1; Giant Axonal Neuropathy (Gigaxonin);
Function
Probable cytoskeletal component that directly or indirectly plays an important role in neurofilament architecture. May act as a substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival.
Biological Process
Cytoskeleton organization Source: InterPro
Protein ubiquitination Source: UniProtKB
Cellular Location
Cytoskeleton; Cytoplasm
Involvement in disease
Giant axonal neuropathy 1, autosomal recessive (GAN1):
A severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. Axonal loss and the presence of giant axonal swellings filled with neurofilaments on nerve biopsies are the hallmarks of this neurodegenerative disorder.
PTM
Ubiquitinated by E3 ubiquitin ligase complex formed by CUL3 and RBX1 and probably targeted for proteasome-independent degradation.
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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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