Metabolism Antibodies 1
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
Description
| Antibody Name: | Anti-IVD Antibody |
| Antibody SKU: | CAB15281 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 33-200 of human IVD (NP_002216.2). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:200 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | MCF7, Mouse heart, Mouse kidney, Mouse liver |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 33-200 of human IVD (NP_002216.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | 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 |
| Gene ID: | 3712 |
| Uniprot: | P26440 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 42kDa/46kDa |
| Observed MW: | 42kDa |
| Synonyms: | IVD, ACAD2 |
| Background: | 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 |
 | Western blot analysis of extracts of various cell lines, using IVD antibody (CAB15281) 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 Basic Kit (RM00020). Exposure time: 1S. |
 | Immunohistochemistry of paraffin-embedded rat liver using IVD antibody (CAB15281) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human colon carcinoma using IVD antibody (CAB15281) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse kidney using IVD antibody (CAB15281) at dilution of 1:100 (40x lens). |
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