IQCB1 Antibody, FITC conjugated (PACO55920)
- SKU:
- PACO55920
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | IQCB1 Antibody, FITC conjugated (PACO55920) |
抗体コード: | PACO55920 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human IQ calmodulin-binding motif-containing protein 1 protein (445-598AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | Involved in ciliogenesis. The function in an early step in cilia formation depends on its association with CEP290/NPHP6. Involved in regulation of the BBSome complex integrity, specifically for presence of BBS2 and BBS5 in the complex, and in ciliary targeting of selected BBSome cargos. May play a role in controlling entry of the BBSome complex to cilia possibly implicating CEP290/NPHP6. |
シノニム: | IQ calmodulin-binding motif-containing protein 1 (Nephrocystin-5) (p53 and DNA damage-regulated IQ motif protein) (PIQ), IQCB1, KIAA0036 NPHP5 |
UniProt Protein Function: | IQCB1: Involved in ciliogenesis. Defects in IQCB1 are the cause of Senior-Loken syndrome type 5 (SLSN5). SLSN is a renal-retinal disorder, characterized by progressive wasting of the filtering unit of the kidney (nephronophthisis), with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Chromosomal Location of Human Ortholog: 3q13.33|3q21.1 Cellular Component: centrosome; cytosol; intercellular bridge; microtubule cytoskeleton; nucleoplasm; photoreceptor connecting cilium Molecular Function:calmodulin binding; enzyme binding; protein binding Biological Process: cilium biogenesis; maintenance of organ identity; photoreceptor cell maintenance Disease: Senior-loken Syndrome 5 |
NCBI Summary: | This gene encodes a nephrocystin protein that interacts with calmodulin and the retinitis pigmentosa GTPase regulator protein. The encoded protein has a central coiled-coil region and two calmodulin-binding IQ domains. It is localized to the primary cilia of renal epithelial cells and connecting cilia of photoreceptor cells. The protein is thought to play a role in ciliary function. Defects in this gene result in Senior-Loken syndrome type 5. Alternative splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 6. [provided by RefSeq, Jan 2016] |
UniProt Code: | Q15051 |
NCBI GenInfo Identifier: | 3123054 |
NCBI Gene ID: | 9657 |
NCBI Accession: | Q15051.1 |
UniProt Secondary Accession: | Q15051,Q3KS08, Q3KS09, Q5DKQ7, Q8NI79, Q9BS08, |
UniProt Related Accession: | Q15051 |
Molecular Weight: | 34,132 Da |
NCBI Full Name: | IQ calmodulin-binding motif-containing protein 1 |
NCBI Synonym Full Names: | IQ motif containing B1 |
NCBI Official Symbol: | IQCB1 |
NCBI Official Synonym Symbols: | PIQ; NPHP5; SLSN5 |
NCBI Protein Information: | IQ calmodulin-binding motif-containing protein 1 |
UniProt Protein Name: | IQ calmodulin-binding motif-containing protein 1 |
UniProt Synonym Protein Names: | Nephrocystin-5; p53 and DNA damage-regulated IQ motif protein; PIQ |
Protein Family: | IQ calmodulin-binding motif-containing protein |
UniProt Gene Name: | IQCB1 |
UniProt Entry Name: | IQCB1_HUMAN |