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

Name: Mouse Anti-GAN Recombinant Antibody (4G7)
SKU: CBMAB-G1885-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

4G7

Application

ELISA, IF, WB

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.

More Infomation

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

8139

UniProt ID

Q9H2C0

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

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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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