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α-ケトグルタル酸尿症
に定義されています α-ケトグルタル酸尿症
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私はありますか? 'α-ケトグルタル酸尿症'?
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α-ケトグルタル酸尿症 および 尿クロマトグラフィー
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α-ケトグルタル酸尿症 および 尿のpH
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α-ケトグルタル酸尿症 および 尿有機酸
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α-ケトグルタル酸尿症 および 血清有機酸類
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治療の手順の検索結果 'α-ケトグルタル酸尿症'
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- alpha-ketoglutaric aciduria
Studies on alpha-ketoglutaric aciduria in type I glycogenosis.
Studies on alpha-ketoglutaric aciduria in type I glycogenosis. ... alpha-Ketoglutaric aciduria was not improved by the acute or prolonged administration of a large dose of ...
http://www.ncbi.nlm.nih.gov/pubmed/6936873
www.ncbi.nlm.nih.gov
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summary
Amish lethal microcephaly - Genetics Home Reference
A compound called alpha-ketoglutaric acid can be detected in their urine (alpha-ketoglutaric aciduria), and during episodes of viral illness they ...
http://ghr.nlm.nih.gov/condition=amishlethalmicrocephaly
ghr.nlm.nih.gov
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summary
Amish lethal microcephaly - Medpedia
A compound called alpha-ketoglutaric acid can be detected in their urine (alpha-ketoglutaric aciduria), and during episodes of viral illness they ...
http://wiki.medpedia.com/Amish_lethal_microcephaly
wiki.medpedia.com
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summary
Amish Lethal Microcephaly - Genetic Information - AccessDNA
A compound called alpha-ketoglutaric acid can be detected in their urine (called alpha-ketoglutaric aciduria), and during episodes of viral illness ...
http://www.accessdna.com/condition/Amish_Lethal_Microcephaly/33
www.accessdna.com
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summary
Multiple alpha-keto aciduria in Microtus montanus chronically ...
... excreted in urine greatly elevated quantities of not only the aromatic alpha-keto acids, phenylpyruvic and 4-hydroxyphenylpyruvic acids, but also two...
http://www.medscape.com/medline/abstract/3902349
www.medscape.com
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summary
List of Conditions | NeurometPlus
Alpha-Ketoglutaric aciduria. Alpha-ketoglutaric acid dehydrogenase ... 3-Methylglutaconic aciduria with normal hydratase (type 4 or special variants), 4a ...
http://www.neurometplus.com/list-of-conditions
www.neurometplus.com
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summary
Orphanet: Oxoglutaricaciduria
Oxoglutaric aciduria is caused by deficiency of alpha ketoglutarate dehydrogenase ... of the diagnosis, as alpha ketoglutaric aciduria is a very common non ...
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=31
www.orpha.net
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summary
alpha-linolenic acid - definition of alpha-linolenic acid by ...
Definition of alpha-linolenic acid in the Online Dictionary. Meaning of alpha-linolenic acid. Pronunciation of alpha-linolenic acid. ...
http://www.thefreedictionary.com/alpha-linolenic+acid
www.thefreedictionary.com
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summary
Metabolic Disorder Screening
Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) ... Valproate glucuronide. X ---Adipic acid. X ---Alpha-ketoglutaric acid. X ...
http://biochemweb.slu.edu/msl/disorders.html
biochemweb.slu.edu
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summary
MetaCyc branched-chain alpha-keto acid dehydrogenase complex
Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup urine disease due to a single enzyme defect: dihydrolipoyl ...
http://biocyc.org/META/new-image?object=CPLX-7050
biocyc.org
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summary
Identification of two mutations in a compound heterozygous ...
Using the E1[alpha] bands as internal controls, the relative ... lactic acidosis, [alpha]-ketoglutaric aciduria and variant form of maple syrup urine ...
http://hmg.oxfordjournals.org/cgi/content/full/5/12/1925
hmg.oxfordjournals.org
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summary
Mendelian Inheritance in Man Document Reader
Blood pyruvate, lactate, alpha-ketoglutarate, and branched-chain amino acids ... lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup ...
http://www.angis.org.au/bin/Databases/BIRX/birx_doc?phtomim+246900
www.angis.org.au
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summary
DLE Ácidos Orgânicos
al Aqeel, A.; Rashed, M.; Ozand, P. T., et al. A new patient with alpha-ketoglutaric aciduria and progressive extrapyramidal tract disease. ...
http://www.dle.com.br/produtos/ao/referencias.htm
www.dle.com.br
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summary
Anaplerotic Therapy in Propionic Acidemia - Full Text View ...
Ornithine alpha-ketoglutarate, glutamine, and citrate are commonly used as ... for: Glutamine Ornithine Citric acid alpha-Ketoglutaric acid Sodium Citrate ...
http://clinicaltrials.gov/ct2/show/NCT00645879
clinicaltrials.gov
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summary
HMDB: Showing D-Glucose (HMDB00122)
Kodama H, Okada S, Inui K, Yutaka T, Yabuuchi H: Studies on alpha-ketoglutaric aciduria in type I glycogenosis. Tohoku J Exp Med. 1980 Aug;131(4):347-53. [PubMed ] ...
http://hmdb.ca/metabolites/HMDB00122
hmdb.ca
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summary
glutarate: Definition from Answers.com
glutarate ( ?glüd??r?t ) ( biochemistry ) The salt or ester of glutaric acid. ... structure of alpha-keto-glutaric acid? What is glutaric aciduria? Post a question ...
http://www.answers.com/topic/glutarate
www.answers.com
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summary
Knockout of Slc25a19 causes mitochondrial thiamine ...
... retards brain development and leads to alpha-ketoglutaric aciduria. Previous data suggested that SLC25A19, ... mouse. Thiamine Pyrophosphate. alpha-ketoglutaric acid. Grant Support: ...
http://www.ncbi.nlm.nih.gov/pubmed/17035501
www.ncbi.nlm.nih.gov
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summary
alpha-lipoprotein - definition of alpha-lipoprotein by the ...
Pronunciation of alpha-lipoprotein. Translations of alpha-lipoprotein. alpha-lipoprotein synonyms, alpha-lipoprotein antonyms. Information about alpha ...
http://www.thefreedictionary.com/alpha-lipoprotein
www.thefreedictionary.com
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summary
Alphabetical index - A Encyclopedia for biotech, medicine ...
Alphabetical index - A of Bionity.COM's encyclopedia for biotech, medicine, biology & life sciences. Facts about biochemists, proteins, lab techniques, receptors, ...
http://www.bionity.com/lexikon/e/Articlelist/A/
www.bionity.com
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summary
Encyclopedia of chemistry. Alphabetical index - A. Lexicon ...
Chemie.DE's encyclopedia for chemistry, analytics, pharmaceutics & material sciences. ... Alpha-ketoglutaric acid. Alpha-linolenic acid. Alpha-mannosidosis. Alpha-Methyltryptamine ...
http://www.chemie.de/lexikon/e/Articlelist/A/
www.chemie.de
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summary
Search results
alpha-Ketobutyric acid. Alpha-ketoglutaric acid. Alpha-ketoisocaproic ... Branched-chain alpha-keto acid dehydrogenase complex. Branched-chain amino acids. Branched ...
http://www.definethis.org/word/Guanylic_acid.html
www.definethis.org
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summary
HMDB: Showing Oxoglutaric acid (HMDB00208)
Alpha-ketoglutaric acid is an important biological compound and is a key intermediate in the Krebs cycle. ... Oxalosis I; Glycolic aciduria; Alanine-glyoxylate aminotransferase ...
http://www.hmdb.ca/metabolites/HMDB00208
www.hmdb.ca
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summary
MEHMO - mental retardation, epileptic seizures, hypogonadism ...
SLC25A19 mutations cause Amish lethal microcephaly ( MCPHA), which markedly retards brain development and leads to alpha-ketoglutaric aciduria. Paper-12288113. ...
http://www.ihop-net.org/UniPub/iHOP/gs/93831.html
www.ihop-net.org
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summary
????? : KMLE ?? ?? ?? - ????, ????, ????, ????, ??/??? ??
???????????(hydroxyglutaric aciduria),7. Segawa?(????? ???? ? ??),8 ? ... ??-???????(alpha-ketoglutaric acid)? ?? ????? ?? ??? ?? ??. ???? ?? NADH ??? ??? ??? ?? ????? ???? ??? ...
http://www.kmle.co.kr/search.php?Search=%B1%DB%B7%E7%C5%B8%B8%A3%BB%EA
www.kmle.co.kr
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summary
Mitochondrial encephalomyopathy with elevated methylmalonic ...
... such as succinic acid, citric acid and alpha-ketoglutaric acid. ... aciduria and 3-methylcrotonyl-CoA and propionyl-CoA carboxylase deficiencies in two unrelated patients: a new ...
http://brain.oxfordjournals.org/cgi/content/full/130/3/853
brain.oxfordjournals.org
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summary
Encyclopedia of chemistry. Alphabetical index - A. Lexicon ...
ChemEurope.COM's encyclopedia for chemistry, analytics, pharmaceutics & material sciences. Facts about ... Alpha-ketoglutaric acid. Alpha-linolenic acid. Alpha-mannosidosis. Alpha ...
http://www.chemeurope.com/lexikon/e/Articlelist/A/
www.chemeurope.com
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summary
Plant Succinic Semialdehyde Dehydrogenase. Cloning ...
Email this article to a friend. Similar articles in this journal ... A Novel {alpha}-Ketoglutaric Semialdehyde Dehydrogenase: EVOLUTIONARY INSIGHT INTO AN ...
http://www.plantphysiol.org/cgi/content/full/121/2/589
www.plantphysiol.org
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summary
World J Gastroenterol
A study on a-ketoadipic aciduria by gas chromatographic-mass spectrometry ... and lysine metabolism in alpha aminoadipic aciduria.Am J Med Genet,1980; ...
http://www.wjgnet.com/1007-9327/6/766.asp
www.wjgnet.com
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summary
" What is Essential fatty acid deficiency
Essential fatty acid deficiency results in a dermatitis similar to that seen in zinc or ... and transamination of glutamic acid to alpha-ketoglutaric acid are both impaired in ...
http://medicine.all-details.com/what-is-essential-fatty-acid-deficiency
medicine.all-details.com
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summary
Mitochondrial Disorders
Expression of clock genes: BMAL1 (ARNTL) & Rev-erb-alpha (NR1D1) ... 2-Oxoadipic aciduria & 2-Aminoadipic aciduria. Onset: 2 years. Organic acidemia. Episodes of ...
http://neuromuscular.wustl.edu/mitosyn.html
neuromuscular.wustl.edu
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summary
References - GABA or aminobutyrate - HORT640 - Metabolic ...
Catabolism of alpha-ketoglutarate by a sucA mutant of Bradyrhizobium japonicum: ... for succinate semialdehyde dehydrogenase (gamma-hydroxybutyric aciduria) ...
http://web.ics.purdue.edu/~drhodes/hort640c/referen/GABA.htm
web.ics.purdue.edu
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summary
SciLink | T HAYASHI
"HYPERFOLIC-ACIDEMIA WITH FORMIMINOGLUTAMIC-ACIDURIA FOLLOWING HISTIDINE LOADING" ... determination of pyruvic acid and alpha-ketoglutaric acid in serum. ...
http://www.scilink.com/profile.action?type=author&pk=36701
www.scilink.com
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summary
Examville.com Biochemistry Review Study Guide
Publish your documents quickly and easily, share them with others, and manage or ... Alpha-ketoglutaric acid is one of the most beneficial supplements for ...
http://www.scribd.com/doc/16975158/Examvillecom-Biochemistry-Review-Study-Guide
www.scribd.com
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summary
aminotransferase : KMLE ?? ?? ?? - ????, ????, ????, ????, ?? ...
Synonym: delta-aminovalerate-alpha-ketoglutarate aminotransferase (26 Jun 1999) 7,8 ... forming alpha-ketoglutaric acid and l-aspartic acid; a diagnostic aid in viral ...
http://www.kmle.co.kr/search.php?Search=aminotransferase
www.kmle.co.kr
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summary
Statistique d'Usage du Serveur Orphanet orphanet.orpha.net ...
... 28 0.02% madelung deformity 28 0.02% mevalonic aciduria 28 ... fisura labial 8 0.01% five alpha reductase deficiency 8 0.01% frontometaphyseal dysplasia ...
http://www.orpha.net/stat/orphanet/search_200706.html
www.orpha.net
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summary
Glutarates :: analysis
... acid and related metabolites in body fluids of patients with glutaric aciduria type 1 ... Generalized aminoaciduria, alpha-aminoadipic aciduria, and saccharopinuria were noted ...
http://lib.bioinfo.pl/meid:79979
lib.bioinfo.pl
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summary
???????????????-??-???
2-Oxoadipic aciduria & 2-Aminoadipic aciduria. Onset: 2 years. Organic acidemia. Episodes of ... Lipoamide dehydrogenase deficiency (?-ketoglutaric dehydrogenase) ...
http://www.bioon.com/Article/pathphysio/79567.shtml
www.bioon.com
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summary
D009930D02Organic ChemicalsOrganic Chemicals (vertaling ...
It stimulates both the alpha- and beta- adrenergic systems, causes systemic ... (vertaling onbekend)Blocker of both alpha- and beta-adrenergic receptors that is ...
http://aureality.midiworld.org/bin/mesh/mesh/mesh-D02.xml
aureality.midiworld.org
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summary
EC 1.2.4.2 - oxoglutarate dehydrogenase (succinyl-transferring)
Information on EC 1.2.4.2 - oxoglutarate dehydrogenase (succinyl-transferring) ... alpha-ketoglutaric acid dehydrogenase. alpha-ketoglutaric dehydrogenase. alpha-KGD. alpha ...
http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.2.4.2
www.brenda-enzymes.info
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summary
Dictionary
ALPHA GLUCOSIDASE. An enzyme which removes the last 1,4-linked alpha-D-glucose residue ... L-AminoAcid+KetoGlutaric Acid = L-Glutamic Acid+KetoAcid corresponding to L-AminoAcid. ...
http://www.chemi-tek.com/dic_lista.asp
www.chemi-tek.com
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summary
MetaCyc 2-oxoglutarate
A novel alpha-ketoglutarate reductase activity of the serA-encoded 3 ... coli K-12 and its possible implications for human 2-hydroxyglutaric aciduria. ...
http://www.ecocyc.com/META/NEW-IMAGE?object=2-KETOGLUTARATE
www.ecocyc.com
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summary
Current News and Information on Amino Acid Transporter
An amino acid transporter is a membrane transport protein that transports amino acids. ... leader Hartnup disease Blue diaper syndrome Alpha-Ketoglutaric acid ...
http://instantmedicalnews.com/1/amino_acids/news/Amino-acid-transporter.html
instantmedicalnews.com
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summary
Escherichia coli K-12 substr. MG1655 2-oxoglutarate
A novel alpha-ketoglutarate reductase activity of the serA-encoded 3 ... coli K-12 and its possible implications for human 2-hydroxyglutaric aciduria. ...
http://biocyc.org/ECOLI/NEW-IMAGE?type=COMPOUND&object=2-KETOGLUTARATE
biocyc.org
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summary
Carbohydrate Dehydrogenases :: isolation & purification
BioInfoBank Library :: Carbohydrate Dehydrogenases :: isolation & purification :: Cellobiose dehydrogenase from the ligninolytic basidiomycete Ceriporiopsis ...
http://lib.bioinfo.pl/meid:38850
lib.bioinfo.pl
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summary
Dictionar Medical en Ro
un dictionar util in domeniul medical Dictionaries ... aciduria - 1. excre?ie a unei urine acide; 2. excre?ie a unei cantit??i anormale de elemente acide. aciduric ...
http://www.scribd.com/doc/25363589/Dictionar-Medical-en-Ro
www.scribd.com
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summary
EC 1.2.1.24 - succinate-semialdehyde dehydrogenase
D-2-hydroxyglutaric aciduria in three patients with proven SSADH ... A novel alpha-ketoglutaric semialdehyde dehydrogenase: evolutionary insight into an alternative pathway of ...
http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.2.1.24
www.brenda-enzymes.info
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summary
Reference to EC No. 1.2.1.24, ID= 656808
Human SSADH deficiency results in 4-hydroxybutyric aciduria, an autosomal recessive disorder due to an ... A novel alpha-ketoglutaric sem ... Azospirillum brasilense. J. Biol. ...
http://www.brenda-enzymes.org/literature/lit.php4?e=1.2.1.24&r=656808
www.brenda-enzymes.org
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summary
iB::W?tek::AUTYZM A INNE CHOROBY
Strona Dzieci Sprawnych Inaczej ... j) Alpha-ketoglutaric aciduria. k) Pyruvic dehydrogenase phosphatase deficiency. 1) Primary hyperoxaluria type I and II ...
http://www.dzieci.org.pl/cgi/forum/ikonboard.cgi?act=ST;f=26;t=8786;st=120
www.dzieci.org.pl
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summary
DLD Gene - GeneCards | DLDH Protein | DLDH Antibody
Complete information for DLD gene (protein-coding), dihydrolipoamide dehydrogenase ... Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of ...
http://www.genecards.org/cgi-bin/carddisp.pl?gene=DLD&pubmed=109
www.genecards.org
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summary
OMIM: 248600
... acidosis, maple syrup urine disease, and ketoglutaric aciduria. E3 activity was reduced. Sakaguchi et al. ... lactic acidosis, alpha-ketoglutaric aciduria and variant form of ...
http://www.genome.jp/dbget-bin/www_bget?omim+248600
www.genome.jp
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summary
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