GAN Antibody (PACO43793)
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주문- SKU:
- PACO43793
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | GAN Antibody (PACO43793) |
Antibody SKU: | PACO43793 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Gigaxonin protein (1-240AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunohistochemistry of paraffin-embedded human breast cancer using PACO43793 at dilution of 1:100. | |
Immunohistochemistry of paraffin-embedded human liver cancer using PACO43793 at dilution of 1:100. |
Background: | 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. |
Synonyms: | Gigaxonin (Kelch-like protein 16), GAN, GAN1 KLHL16 |
UniProt Protein Function: | GAN: Probable cytoskeletal component that directly or indirectly plays an important role in neurofilament architecture. 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. Defects in GAN are the cause of giant axonal neuropathy (GAN). GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons. |
UniProt Protein Details: | Chromosomal Location of Human Ortholog: 16q24.1 Cellular Component: cytoskeleton; cytoplasm Molecular Function:protein binding Biological Process: protein ubiquitination Disease: Giant Axonal Neuropathy 1, Autosomal Recessive |
NCBI Summary: | 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] |
UniProt Code: | Q9H2C0 |
NCBI GenInfo Identifier: | 13626745 |
NCBI Gene ID: | 8139 |
NCBI Accession: | Q9H2C0.1 |
UniProt Secondary Accession: | Q9H2C0,Q8CA72, |
UniProt Related Accession: | Q9H2C0 |
Molecular Weight: | 597 |
NCBI Full Name: | Gigaxonin |
NCBI Synonym Full Names: | gigaxonin |
NCBI Official Symbol: | GAN |
NCBI Official Synonym Symbols: | GAN1; KLHL16 |
NCBI Protein Information: | gigaxonin; kelch-like protein 16; kelch-like family member 16 |
UniProt Protein Name: | Gigaxonin |
UniProt Synonym Protein Names: | Kelch-like protein 16 |
Protein Family: | Gigaxonin |
UniProt Gene Name: | GAN |
UniProt Entry Name: | GAN_HUMAN |