Anti-PHYH Antibody (CAB6304)
- SKU:
- CAB6304
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-PHYH Antibody |
| 抗体コード: | CAB6304 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-338 of human PHYH (NP_006205.1). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:500 - 1:2000 IF 1:10 - 1:100 |
| 反応性: | Human, Mouse |
| ポジティブサンプル: | MCF7, U-937, A-549, HepG2 |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-338 of human PHYH (NP_006205.1). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | MEQL RAAA RLQI VLGH LGRP SAGA VVAH PTSG TISS ASFH PQQF QYTL DNNV LTLE QRKF YEEN GFLV IKNL VPDA DIQR FRNE FEKI CRKE VKPL GLTV MRDV TISK SEYA PSEK MITK VQDF QEDK ELFR YCTL PEIL KYVE CFTG PNIM AMHT MLIN KPPD SGKK TSRH PLHQ DLHY FPFR PSDL IVCA WTAM EHIS RNNG CLVV LPGT HKGS LKPH DYPK WEGG VNKM FHGI QDYE ENKA RVHL VMEK GDTV FFHP LLIH GSGQ NKTQ GFRK AISC HFAS ADCH YIDV KGTS QENI EKEV VGIA HKFF GAEN SVNL KDIW MFRA RLVK GERT NL |
| 遺伝子ID: | 5264 |
| Uniprot: | O14832 |
| セルラーロケーション: | Peroxisome |
| 計算された分子量: | 27kDa/38kDa |
| 観察された分子量: | 38kDa |
| 同義語: | PHYH, LN1, LNAP1, PAHX, PHYH1, RD |
| バックグラウンド: | This gene is a member of the PhyH family and encodes a peroxisomal protein that is involved in the alpha-oxidation of 3-methyl branched fatty acids. Specifically, this protein converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Mutations in this gene have been associated with Refsum disease (RD) and deficient protein activity has been associated with Zellweger syndrome and rhizomelic chondrodysplasia punctata. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. |
| UniProt Protein Function: | PHYH: Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH are a cause of Refsum disease (RD). RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF). Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues. Less constant features are nerve deafness, anosmia, skeletal abnormalities, ichthyosis, cataracts and cardiac impairment. Manifestations of the disease appear in the second or third decade of life. Belongs to the PhyH family. |
| UniProt Protein Details: | Protein type:EC 1.14.11.18; Oxidoreductase Chromosomal Location of Human Ortholog: 10p13 Cellular Component: peroxisomal matrix; peroxisome Molecular Function:cofactor binding; phytanoyl-CoA dioxygenase activity; protein binding Biological Process: fatty acid alpha-oxidation; isoprenoid metabolic process Disease: Refsum Disease, Classic |
| NCBI Summary: | This gene is a member of the PhyH family and encodes a peroxisomal protein that is involved in the alpha-oxidation of 3-methyl branched fatty acids. Specifically, this protein converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Mutations in this gene have been associated with Refsum disease (RD) and deficient protein activity has been associated with Zellweger syndrome and rhizomelic chondrodysplasia punctata. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008] |
| UniProt Code: | O14832 |
| NCBI GenInfo Identifier: | 6093646 |
| NCBI Gene ID: | 5264 |
| NCBI Accession: | O14832.1 |
| UniProt Secondary Accession: | O14832,A8MTS8, B1ALH5, |
| UniProt Related Accession: | O14832 |
| Molecular Weight: | 27,291 Da |
| NCBI Full Name: | Phytanoyl-CoA dioxygenase, peroxisomal |
| NCBI Synonym Full Names: | phytanoyl-CoA 2-hydroxylase |
| NCBI Official Symbol: | PHYH |
| NCBI Official Synonym Symbols: | RD; LN1; PAHX; LNAP1; PHYH1 |
| NCBI Protein Information: | phytanoyl-CoA dioxygenase, peroxisomal |
| UniProt Protein Name: | Phytanoyl-CoA dioxygenase, peroxisomal |
| UniProt Synonym Protein Names: | Phytanic acid oxidase; Phytanoyl-CoA alpha-hydroxylase; PhyH |
| Protein Family: | Phytanoyl-CoA dioxygenase |
| UniProt Gene Name: | PHYH |
| UniProt Entry Name: | PAHX_HUMAN |
 | Western blot analysis of extracts of various cell lines, using PHYH antibody (CAB6304) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s. |
 | Immunofluorescence analysis of U2OS cells using PHYH antibody (CAB6304). Blue: DAPI for nuclear staining. |
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