Anti-IVD Antibody (CAB15281)
- SKU:
- CAB15281
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-IVD Antibody |
抗体コード: | CAB15281 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 33-200 of human IVD (NP_002216.2). |
申し込み: | WB IHC |
推奨希釈: | WB 1:200 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | MCF7, Mouse heart, Mouse kidney, Mouse liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 33-200 of human IVD (NP_002216.2). |
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: | HSLL PVDD AING LSEE QRQL RQTM AKFL QEHL APKA QEID RSNE FKNL REFW KQLG NLGV LGIT APVQ YGGS GLGY LEHV LVME EISR ASGA VGLS YGAH SNLC INQL VRNG NEAQ KEKY LPKL ISGE YIGA LAMS EPNA GSDV VSMK LKAE KKGN HYIL NGNK FWIT |
遺伝子ID: | 3712 |
Uniprot: | P26440 |
セルラーロケーション: | Mitochondrion matrix |
計算された分子量: | 42kDa/46kDa |
観察された分子量: | 42kDa |
同義語: | IVD, ACAD2 |
バックグラウンド: | Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. |
UniProt Protein Function: | IVD: Defects in IVD are the cause of isovaleric acidemia (IVA). IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Belongs to the acyl-CoA dehydrogenase family. |
UniProt Protein Details: | Protein type:EC 1.3.8.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 15q14-q15 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix Molecular Function:isovaleryl-CoA dehydrogenase activity; FAD binding Biological Process: leucine catabolic process; branched chain family amino acid catabolic process Disease: Isovaleric Acidemia |
NCBI Summary: | Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009] |
UniProt Code: | P26440 |
NCBI GenInfo Identifier: | 226958414 |
NCBI Gene ID: | 3712 |
NCBI Accession: | NP_001152980.1 |
UniProt Secondary Accession: | P26440,Q53XZ9, Q96AF6, B2RCV5, B3KVI7, J3KR54, |
UniProt Related Accession: | P26440 |
Molecular Weight: | 46,319 Da |
NCBI Full Name: | isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 |
NCBI Synonym Full Names: | isovaleryl-CoA dehydrogenase |
NCBI Official Symbol: | IVD |
NCBI Official Synonym Symbols: | ACAD2 |
NCBI Protein Information: | isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase |
UniProt Protein Name: | Isovaleryl-CoA dehydrogenase, mitochondrial |
Protein Family: | Isovaleryl-CoA dehydrogenase |
UniProt Gene Name: | IVD |
UniProt Entry Name: | IVD_HUMAN |
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