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Anti-IVD Antibody (CAB15281)

SKU:
CAB15281
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism
  • Metabolism Antibodies 1 Anti-IVD Antibody CAB15281
  • Metabolism Antibodies 1 Anti-IVD Antibody CAB15281
  • Metabolism Antibodies 1 Anti-IVD Antibody CAB15281
  • Metabolism Antibodies 1 Anti-IVD Antibody CAB15281
€139 - €419
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Description

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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
Anti-IVD Antibody (CAB15281)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.
Anti-IVD Antibody (CAB15281)Immunohistochemistry of paraffin-embedded rat liver using IVD antibody (CAB15281) at dilution of 1:100 (40x lens).
Anti-IVD Antibody (CAB15281)Immunohistochemistry of paraffin-embedded human colon carcinoma using IVD antibody (CAB15281) at dilution of 1:100 (40x lens).
Anti-IVD Antibody (CAB15281)Immunohistochemistry of paraffin-embedded mouse kidney using IVD antibody (CAB15281) at dilution of 1:100 (40x lens).

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