Anti-SUOX Antibody (CAB14258)
- SKU:
- CAB14258
- Product type:
- Antibody
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-SUOX Antibody |
抗体コード: | CAB14258 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 376-545 of human SUOX (NP_000447.2). |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Mouse |
ポジティブサンプル: | Mouse brain, Mouse heart |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 376-545 of human SUOX (NP_000447.2). |
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: | RHVK WLGR VSVQ PEES YSHW QRRD YKGF SPSV DWET VDFD SAPS IQEL PVQS AITE PRDG ETVE SGEV TIKG YAWS GGGR AVIR VDVS LDGG LTWQ VAKL DGEE QRPR KAWA WRLW QLKA PVPA GQKE LNIV CKAV DDGY NVQP DTVA PIWN LRGV LSNA WHRV HVYV SP |
遺伝子ID: | 6821 |
Uniprot: | P51687 |
セルラーロケーション: | Mitochondrion intermembrane space |
計算された分子量: | 60kDa |
観察された分子量: | 60kDa |
同義語: | SUOX |
バックグラウンド: | Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. |
UniProt Protein Function: | SUOX: Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD); also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age. |
UniProt Protein Details: | Protein type:Mitochondrial; Energy Metabolism - sulfur; Oxidoreductase; EC 1.8.3.1 Chromosomal Location of Human Ortholog: 12q13.2 Cellular Component: mitochondrial matrix; mitochondrial intermembrane space Molecular Function:molybdopterin cofactor binding; electron carrier activity; molybdenum ion binding; heme binding; sulfite oxidase activity Biological Process: sulfur amino acid metabolic process; sulfur amino acid catabolic process Disease: Sulfocysteinuria |
NCBI Summary: | Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008] |
UniProt Code: | P51687 |
NCBI GenInfo Identifier: | 152031695 |
NCBI Gene ID: | 6821 |
NCBI Accession: | P51687.2 |
UniProt Related Accession: | P51687 |
Molecular Weight: | 545 |
NCBI Full Name: | Sulfite oxidase, mitochondrial |
NCBI Synonym Full Names: | sulfite oxidase |
NCBI Official Symbol: | SUOX |
NCBI Protein Information: | sulfite oxidase, mitochondrial |
UniProt Protein Name: | Sulfite oxidase, mitochondrial |
Protein Family: | Sulfite oxidase |
UniProt Gene Name: | SUOX |
UniProt Entry Name: | SUOX_HUMAN |
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