Anti-ACOX1 Antibody (CAB8091)
- SKU:
- CAB8091
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Description
抗体名: | ACOX1 Rabbit Polyclonal Antibody |
抗体コード: | CAB8091 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-270 of human ACOX1 (NP_004026.2). |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:100 - 1:200 IF 1:50 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | NCI-H460, Mouse liver, Mouse kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-270 of human ACOX1 (NP_004026.2). |
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: | MNPD LRRE RDSA SFNP ELLT HILD GSPE KTRR RREI ENMI LNDP DFQH EDLN FLTR SQRY EVAV RKSA IMVK KMRE FGIA DPDE IMWF KNFV HRGR PEPL DLHL GMFL PTLL HQAT AEQQ ERFF MPAW NLEI IGTY AQTE MGHG THLR GLET TATY DPET QEFI LNSP TVTS IKWW PGGL GKTS NHAI VLAQ LITK GKCY GLHA FIVP IREI GTHK PLPG ITVG DIGP KFGY DEID NGYL KMDN HRIP RENM LMKY AQVK PDGT YVKP LS |
遺伝子ID: | 51 |
Uniprot: | Q15067 |
セルラーロケーション: | Peroxisome |
計算された分子量: | 70kDa/74kDa |
観察された分子量: | 74kDa |
同義語: | ACOX1, ACOX, PALMCOX, SCOX |
バックグラウンド: | The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified. |
UniProt Protein Function: | ACOX1: Catalyzes the desaturation of acyl-CoAs to 2-trans- enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy- palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl- CoA. Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. Belongs to the acyl-CoA oxidase family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:EC 1.3.3.6; Oxidoreductase; Lipid Metabolism - unsaturated fatty acid biosynthesis; Lipid Metabolism - fatty acid; Lipid Metabolism - alpha-linolenic acid Chromosomal Location of Human Ortholog: 17q25.1 Cellular Component: intracellular membrane-bound organelle; membrane; nucleolus; nucleoplasm; nucleus; peroxisomal matrix; peroxisome; plasma membrane Molecular Function:acyl-CoA binding; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; electron carrier activity; PDZ domain binding; protein N-terminus binding; receptor binding Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid oxidation; generation of precursor metabolites and energy; lipid metabolic process; prostaglandin metabolic process; very-long-chain fatty acid metabolic process Disease: Peroxisomal Acyl-coa Oxidase Deficiency |
NCBI Summary: | The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q15067 |
NCBI GenInfo Identifier: | 126302511 |
NCBI Gene ID: | 51 |
NCBI Accession: | Q15067.3 |
UniProt Secondary Accession: | Q15067,Q12863, Q15068, Q15101, Q16131, Q7Z3W5, Q9UD31 A8K6X8, A8KAA0, B4DK61, F5GYQ8, |
UniProt Related Accession: | Q15067 |
Molecular Weight: | 70,136 Da |
NCBI Full Name: | Peroxisomal acyl-coenzyme A oxidase 1 |
NCBI Synonym Full Names: | acyl-CoA oxidase 1 |
NCBI Official Symbol: | ACOX1 |
NCBI Official Synonym Symbols: | ACOX; SCOX; PALMCOX |
NCBI Protein Information: | peroxisomal acyl-coenzyme A oxidase 1 |
UniProt Protein Name: | Peroxisomal acyl-coenzyme A oxidase 1 |
UniProt Synonym Protein Names: | Palmitoyl-CoA oxidase; Straight-chain acyl-CoA oxidase; SCOX |
Protein Family: | Peroxisomal acyl-coenzyme A oxidase |
UniProt Gene Name: | ACOX1 |
UniProt Entry Name: | ACOX1_HUMAN |