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isovaleryl coa dehydrogenase deficiency - inherited multisystem disease
Definition for isovaleryl CoA dehydrogenase deficiency
http://ghr.nlm.nih.gov/gene=ivd
some common symptoms
convulsions
fatigue
poor appetite
vomiting
some related diagnoses
1 - 7 of
64
diagnoses
alcoholism
drugs and toxins
human immunodeficiency virus infections
subdural hematoma
substance abuse
intracranial tumor
hydrocephalus
more
do i have 'isovaleryl CoA dehydrogenase deficiency'?
In addition the following diagnostic tests might be need to help verify the diagnosis:
diagnostic test search results for 'isovaleryl coa dehydrogenase deficiency'
1 - 5 of
16
diagnostic tests
Molecular Genetic Studies
isovaleryl CoA dehydrogenase deficiency and Molecular Genetic Studies
Plasma amino acids
isovaleryl CoA dehydrogenase deficiency and Plasma amino acids
urinary isovaleric acid
isovaleryl CoA dehydrogenase deficiency and urinary isovaleric acid
urinary isovalerylglycin
isovaleryl CoA dehydrogenase deficiency and urinary isovalerylglycin
urine amino acids
isovaleryl CoA dehydrogenase deficiency and urine amino acids
Blood, Platelet Count
Platelet Count
isovaleryl CoA dehydrogenase deficiency and Blood, Platelet Count
Complete Blood Count
isovaleryl CoA dehydrogenase deficiency and Complete Blood Count
EEG - Electroencephalogram
isovaleryl CoA dehydrogenase deficiency and EEG - Electroencephalogram
serum isovaleric acid
isovaleryl CoA dehydrogenase deficiency and serum isovaleric acid
Brain MRI
isovaleryl CoA dehydrogenase deficiency and Brain MRI
Amniocentesis
isovaleryl CoA dehydrogenase deficiency and Amniocentesis
Serum Organic Acids
isovaleryl CoA dehydrogenase deficiency and Serum Organic Acids
Urine chromatography
isovaleryl CoA dehydrogenase deficiency and Urine chromatography
serum glycine
isovaleryl CoA dehydrogenase deficiency and serum glycine
urine Organic Acids
isovaleryl CoA dehydrogenase deficiency and urine Organic Acids
urine pH
isovaleryl CoA dehydrogenase deficiency and urine pH
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therapeutic procedures for 'isovaleryl CoA dehydrogenase deficiency'?
Managing or treating a diseases should always be determined by a patients health care provider. Below is a list of associated therapeutic procedures or medications for isovaleryl CoA dehydrogenase deficiency:
therapeutic procedure search results for 'isovaleryl coa dehydrogenase deficiency'
1 - 5 of
6
therapeutic procedures
rankings are computer generated. please consult your health care provider.
Protein restriction
isovaleryl CoA dehydrogenase deficiency and Protein restriction
Avoid fasting
isovaleryl CoA dehydrogenase deficiency and Avoid fasting
supplements of carnitine and glycine
isovaleryl CoA dehydrogenase deficiency and supplements of carnitine and glycine
avoid leucine in diet
isovaleryl CoA dehydrogenase deficiency and avoid leucine in diet
Nutritional counseling
isovaleryl CoA dehydrogenase deficiency and Nutritional counseling
diet modification
isovaleryl CoA dehydrogenase deficiency and diet modification
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medications search results for 'isovaleryl coa dehydrogenase deficiency'
rankings are computer generated. please consult your health care provider.
web search results for isovaleryl coa dehydrogenase deficiency
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1 - 50
- isovaleryl CoA dehydrogenase deficiency
Acyl CoA dehydrogenase - Wikipedia, the free encyclopedia
Medium Chain Acyl-CoA Dehydrogenase Deficiency." Archives of Disease in Childhood. ... SRD5A2) · Glutaryl-CoA dehydrogenase · Isovaleryl coenzyme A dehydrogenase ...
http://en.wikipedia.org/wiki/Acyl_CoA_dehydrogenase
en.wikipedia.org
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summary
Different spectrum of mutations of isovaleryl-CoA dehydrogenase (IVD ...
... metabolism that is caused by a deficiency of isovaleryl-CoA dehydrogenase (IVD) ... Isovaleryl-CoA Dehydrogenase. Grant Support: R01DK45482/DK/NIDDK NIH ...
http://www.ncbi.nlm.nih.gov/pubmed/17576084
www.ncbi.nlm.nih.gov
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summary
IVD - isovaleryl Coenzyme A dehydrogenase - Genetics Home Reference
Isovaleryl-CoA dehydrogenase helps process a particular amino ... deficiency of isovaleryl-CoA dehydrogenase, which mediates formation of 3-methylcrotonate ...
http://ghr.nlm.nih.gov/gene=ivd
ghr.nlm.nih.gov
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summary
Characterization of molecular defects in isovaleryl-CoA dehydrogenase ...
Isovaleryl-CoA dehydrogenase (IVD) is a homotetrameric ... Oxidoreductases/deficiency. Oxidoreductases/genetics* Oxidoreductases/isolation & purification ...
http://www.ncbi.nlm.nih.gov/pubmed/9665741
www.ncbi.nlm.nih.gov
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summary
Isovaleryl coenzyme A dehydrogenase - Wikipedia, the free encyclopedia
A deficiency of this enzyme causes isovaleric acidemia. [edit] External links. MeSH Isovaleryl-CoA+Dehydrogenase. File:AngiotensinCE-1O8A.png ...
http://en.wikipedia.org/wiki/Isovaleryl_coenzyme_A_dehydrogenase
en.wikipedia.org
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summary
Structural organization of the human isovaleryl-CoA dehydrogenase gene.
Isovaleryl-CoA dehydrogenase (IVD) catalyzes the third step in leucine catabolism. The genetic deficiency of IVD causes isovaleric acidemia. ...
http://www.ncbi.nlm.nih.gov/pubmed/8468053
www.ncbi.nlm.nih.gov
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summary
Amino Acid and Organic Acid Metabolism Disorders: Inherited Disorders ...
Deficiency of this dehydrogenase results in isovaleric acidemia, also known as " ... Deficiency of propionyl CoA carboxylase, the enzyme responsible for metabolizing ...
http://www.merck.com/mmpe/print/sec19/ch296/ch296c.html
www.merck.com
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summary
WebMD Health A-Z - Find reliable health and medical information on ...
Isovaleric Acid CoA Dehydrogenase Defici... Isovaleric Acidaemia. Isovalericacidemia. Isovaleryl CoA Carboxylase Deficiency. Itermittent Ataxia With Pyruvate ...
http://www.webmd.com/a-to-z-guides/health-topics/ip-it.htm
www.webmd.com
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summary
Isovaleric acidemia - Genetics Home Reference
Isovaleric acid-CoA dehydrogenase deficiency. Isovaleryl-CoA dehydrogenase deficiency ... deficiency ; dehydrogenase ; enzyme ; failure to thrive ; fasting ; gene ; ...
http://ghr.nlm.nih.gov/condition=isovalericacidemia
ghr.nlm.nih.gov
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summary
IVD - NextBio
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 ...
http://www.nextbio.com/b/home/home.nb?q=IVD
www.nextbio.com
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summary
Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency -- GeneReviews ...
... to MCAD are isovaleryl-CoA dehydrogenase (IVD), 2-methyl branched-chain acyl-CoA ... ACYL-CoA DEHYDROGENASE, MEDIUM-CHAIN, DEFICIENCY OF. 607008 ...
http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=mcad
www.ncbi.nlm.nih.gov
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summary
2,4 Dienoyl-CoA reductase - Wikipedia, the free encyclopedia
Mutations in the DECR1 gene may result in 2,4 Dienoyl-CoA reductase deficiency.[5] ... SRD5A2) · Glutaryl-CoA dehydrogenase · Isovaleryl coenzyme A dehydrogenase ...
http://en.wikipedia.org/wiki/2,4_Dienoyl-CoA_reductase
en.wikipedia.org
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summary
Food, nutrition, and metabolism - Genetics Home Reference
acyl-CoA dehydrogenase very long chain deficiency see very ... Isovaleryl-CoA dehydrogenase deficiency see isovaleric acidemia. IVA see isovaleric acidemia ...
http://ghr.nlm.nih.gov/conditionCategory=foodnutritionandmetabolism
ghr.nlm.nih.gov
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summary
ISOVALERIC ACIDEMIA - Pedbase.org
deficiency of isovaleryl CoA dehydrogenase in leukocytes or cultured skin fibroblasts ... deficiency of isovaleryl CoA dehydrogenase in cultured chorionic ...
http://pedbase.org/i/isovaleric-acidemia/
pedbase.org
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summary
3-hydroxy-3-methylglutaryl-CoA lyase - Wikipedia, the free encyclopedia
Mutations in the HMGCL gene cause 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. ... · Branched-chain alpha-keto acid dehydrogenase complex · Isovaleryl coenzyme A ...
http://en.wikipedia.org/wiki/3-hydroxy-3-methylglutaryl-CoA_lyase
en.wikipedia.org
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summary
Disorders of Organic Acid Metabolism
... a congenital deficiency of isovaleryl-CoA dehydrogenase, which mediates ... 3-Hydroxyisobutyryl-CoA deacylase deficiency causes a block in valine catabolism ...
http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=bnchm.section.3108
www.ncbi.nlm.nih.gov
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summary
3-Methylcrotonyl-CoA carboxylase deficiency
Isolated methylcrotonyl-CoA carboxylase deficiency (MIM 210200 and 210210) is ... step upstream to MCC (isovaleryl-CoA dehydrogenase) is irreversible, there is no ...
http://www.orpha.net/data/patho/GB/uk-MCC.html
www.orpha.net
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summary
Acidemia, Isovaleric - Yahoo! Health
Isovaleric Acid CoA Dehydrogenase Deficiency. Isovaleric Acidaemia ... It is characterized by a deficiency of the enzyme isovaleryl CoA dehydrogenase. ...
http://health.yahoo.com/blood-overview/acidemia-isovaleric/h...
health.yahoo.com
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summary
Branched-chain alpha-keto acid dehydrogenase complex - Wikipedia, the ...
... and the pyruvate dehydrogenase complex creating acetyl-CoA prior to the citric acid cycle. ... keto acid dehydrogenase complex · Isovaleryl coenzyme A ...
http://en.wikipedia.org/wiki/Branched-chain_alpha-keto_acid_dehydrogenase
en.wikipedia.org
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summary
Molecular characterization of four different classes of mutations in ...
... determined deficiency of isovaleryl-CoA dehydrogenase (IVD), a mitochondrial matrix enzyme. ... Isovaleryl-CoA Dehydrogenase. Molecular Sequence Data ...
http://www.ncbi.nlm.nih.gov/pubmed/2063866
www.ncbi.nlm.nih.gov
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summary
Fatty Acid and Glycerol Metabolism Disorders: Inherited Disorders of ...
Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) ... acid analysis, and glutaryl and isovaleryl and other acylcarnitines in tandem ...
http://www.merck.com/mmpe/print/sec19/ch296/ch296e.html
www.merck.com
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summary
New Descriptors - 2006
DNA Repair-Deficiency Disorders. Dopamine and cAMP-Regulated Phosphoprotein 32 ... 3-Isopropylmalate Dehydrogenase. Isovaleryl-CoA Dehydrogenase. Kangai-1 Protein ...
http://www.nlm.nih.gov/mesh/newd2006.html
www.nlm.nih.gov
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summary
The Organic Acidemias: An Overview -- GeneReviews -- NCBI Bookshelf
Biotin-unresponsive 3-methylcrotonyl -CoA carboxylase deficiency. X. Hypoglycemia ... Isovaleryl CoA dehydrogenase. 243500, 607036. Clinical ...
http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=oa-overview
www.ncbi.nlm.nih.gov
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summary
Isobutyryl-CoA - Wikipedia, the free encyclopedia
Isobutyryl-CoA mutase. Isobutyryl-coenzyme A dehydrogenase deficiency. v ? d ? e ... acid · Isovaleryl-CoA · 3-Methylcrotonyl-CoA · 3-Methylglutaconyl-CoA · HMG-CoA ...
http://en.wikipedia.org/wiki/Isobutyryl-CoA
en.wikipedia.org
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summary
Exon skipping in IVD RNA processing in isovaleric acidemia caused by ...
... a recessive disorder caused by a deficiency of isovaleryl-CoA dehydrogenase (IVD) ... Isovaleryl-CoA Dehydrogenase. Metabolism, Inborn Errors/enzymology ...
http://www.ncbi.nlm.nih.gov/pubmed/10677295
www.ncbi.nlm.nih.gov
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summary
Isovaleric acidemia. Clinical presentation of 6 cases.
Oxidoreductases/deficiency* Oxidoreductases Acting on CH-CH Group Donors* Recurrence ... Isovaleryl-CoA Dehydrogenase. PMID: 9673537 [PubMed - indexed for MEDLINE] ...
http://www.ncbi.nlm.nih.gov/pubmed/9673537
www.ncbi.nlm.nih.gov
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summary
2-Methylbutyryl-CoA - Wikipedia, the free encyclopedia
2-Methylbutyryl-CoA dehydrogenase deficiency. v ? d ? e ... acid · Isovaleryl-CoA · 3-Methylcrotonyl-CoA · 3-Methylglutaconyl-CoA · HMG-CoA ...
http://en.wikipedia.org/wiki/2-Methylbutyryl-CoA
en.wikipedia.org
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summary
NCBI Conserved Domain Search
GCD deficiency leads to a severe neurological disorder in humans. ... Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?INPUT_TY...
www.ncbi.nlm.nih.gov
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summary
Intracellular membrane-bounded organelle - Gene Category - Genetics ...
ERCC6: excision repair cross-complementing rodent repair deficiency, complementation group 6 ... IVD: isovaleryl Coenzyme A dehydrogenase. JPH3: junctophilin 3 ...
http://ghr.nlm.nih.gov/ghr/genesByCategory/show/GO_0005575,G...
ghr.nlm.nih.gov
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summary
R Ensenauer - NextBio
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in ... that corresponds to the isovaleryl-CoA dehydrogenase reaction in the degradation ...
http://www.nextbio.com/b/literature/literature.nb?author=R+Ensenauer
www.nextbio.com
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summary
New Descriptors by Tree Subcategory - 2006
DNA Repair-Deficiency Disorders. Dyslipidemias. Glycogen Storage ... 3-Isopropylmalate Dehydrogenase. Isovaleryl-CoA Dehydrogenase. Ketol-Acid Reductoisomerase ...
http://www.nlm.nih.gov/mesh/newbysub2006.html
www.nlm.nih.gov
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summary
NCBI Conserved Domain Search
GCD deficiency leads to a severe neurological disorder in humans. ... Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?INPUT_TY...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
GCD deficiency leads to a severe neurological disorder in humans. ... Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?INPUT_TY...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... GCD deficiency leads to a severe neurological disorder in humans. ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?SEQUENCE...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... GCD deficiency leads to a severe neurological disorder in humans. ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?SEQUENCE...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... GCD deficiency leads to a severe neurological disorder in humans. ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?SEQUENCE...
www.ncbi.nlm.nih.gov
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summary
High-performance liquid chromatographic separation of acylcarnitines ...
... containing butyryl-, isobutyryl-, isovaleryl-, and 2-methylbutyrylcarnitine. ... acidemia, and medium-chain acyl-CoA dehydrogenase deficiency were performed. ...
http://www.ncbi.nlm.nih.gov/pubmed/2344045
www.ncbi.nlm.nih.gov
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summary
Aldehyde dehydrogenase 4 family, member A1 - Wikipedia, the free ...
... · Branched-chain alpha-keto acid dehydrogenase complex · Isovaleryl coenzyme A dehydrogenase · Methylcrotonyl-CoA carboxylase · Methylglutaconyl-CoA hydratase ...
http://en.wikipedia.org/wiki/Aldehyde_dehydrogenase_4_family,_member_A1
en.wikipedia.org
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summary
Intracellular - Gene Category - Genetics Home Reference
ERCC6: excision repair cross-complementing rodent repair deficiency, complementation group 6 ... IVD: isovaleryl Coenzyme A dehydrogenase. JPH3: junctophilin 3 ...
http://ghr.nlm.nih.gov/GenesByCategory/GO_0005575,GO_0005623...
ghr.nlm.nih.gov
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summary
Methylcrotonyl-CoA carboxylase - Wikipedia, the free encyclopedia
... for MCC can lead to MCC deficiency, an inborn error of metabolism which usually ... · Branched-chain alpha-keto acid dehydrogenase complex · Isovaleryl coenzyme A ...
http://en.wikipedia.org/wiki/Methylcrotonyl-CoA_carboxylase
en.wikipedia.org
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summary
NCBI Conserved Domain Search
GCD deficiency leads to a severe neurological disorder in humans. ... Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?INPUT_TY...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... GCD deficiency leads to a severe neurological disorder in humans. ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?SEQUENCE...
www.ncbi.nlm.nih.gov
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summary
NCBI Conserved Domain Search
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... GCD deficiency leads to a severe neurological disorder in humans. ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/wrpsb.cgi?SEQUENCE...
www.ncbi.nlm.nih.gov
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summary
Orphanet: Isovaleric acidemia
Isovaleric acid CoA dehydrogenase deficiency. Summary ... by a deficit in isovaleryl CoA dehydrogenase which affects leucine metabolism. ...
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=33
www.orpha.net
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summary
Book bnchm
Maple syrup urine disease is caused by a congenital deficiency of reaction 2. ... chain amino acid decarboxylase; 3, isovaleryl-CoA dehydrogenase; 4, glycine ...
http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=bnchm.figgrp.3097
www.ncbi.nlm.nih.gov
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summary
Isovaleryl-CoA dehydrogenase deficiency (search)
http://www.nextbio.com/b/home/generalSearch.nb?q=Isovaleryl-...
www.nextbio.com
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summary
OMIM Update List for November, 2004
607036 ISOVALERYL CoA DEHYDROGENASE; IVD ... 609016 LONG-CHAIN 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY ... HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY. 609021 ...
http://www.ncbi.nlm.nih.gov/Omim/dispmonthly.cgi?11.2004
www.ncbi.nlm.nih.gov
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summary
OMIM Update List for April, 2005
201450 ACYL-CoA DEHYDROGENASE, MEDIUM-CHAIN, DEFICIENCY OF ... 607036 ISOVALERYL-CoA DEHYDROGENASE; IVD. 607108 PAIRED BOX GENE 6; PAX6 ...
http://www.ncbi.nlm.nih.gov/Omim/dispmonthly.cgi?4.2005
www.ncbi.nlm.nih.gov
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summary
Octanoyl-coenzyme A(Capryloyl-CoA) - NextBio
... active acyl-CoA dehydrogenase deficiency acyl-CoA hydrolase activity acyl ... characterization of isovaleryl-CoA dehydrogenase (LiuA) of Pseudomonas ...
http://www.nextbio.com/b/home/home.nb?q=Capryloyl-CoA&id=545...
www.nextbio.com
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summary
NCBI CDD cd01156
Isovaleryl-CoA dehydrogenase (IVD) is an is an acyl-CoA dehydrogenase, which ... a congenital deficiency of isovaleryl-CoA dehydrogenase, which mediates ...
http://www.ncbi.nlm.nih.gov/Structure/cdd/cddsrv.cgi?ascbin=...
www.ncbi.nlm.nih.gov
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summary
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