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893 Possible Causes for A, activity, coenzyme, decarboxylase, glutaconyl

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  • Isovaleric Acidemia

    The deficiency of the decarboxylase leads to an accumulation of the branched-chain amino acids and their . . .[nejm.org] Residual activities of the nine isovaleric acidemia lines tested ranged from 0 to 0.67 pmol 3H2O/min/mg protein (controls 19.4 /- 8.0).[ncbi.nlm.nih.gov] Abstract Isovaleric acidemia, resulting from isovaleryl-coenzyme A dehydrogenase deficiency, is associated with marked reduction of free carnitine in both plasma and urine[ncbi.nlm.nih.gov] ReactionCatalysis2080 ACTIVATION 388543 ChemSpider HMDB00202 HMDB HMDB00687 HMDB 388307 ChemSpider SMPDB 4.0 2.0 22833590 PubChem-compound C00141 KEGG Compound TRANS-3-METHYL-GLUTACONYL-COA[smpdb.ca] Amino Acids Urine Organic Acids (orotic) Arginosuccinic Aciduria or Arginosuccinate Lyase Deficiency (ASA) Plasma Amino Acids Urine Organic Acids (orotic) Aromatic L-amino Decarboxylase[baylorhealth.edu]

    Missing: A
  • Hepatoerythropoietic Porphyria

    Abstract Hepatoerythropoietic porphyria (HEP) is due to a marked deficiency of uroporphyrinogen (URO) decarboxylase, a cytosolic enzyme in the heme biosynthetic pathway.[ncbi.nlm.nih.gov] Erythrocyte UROD activity was 42% of normal and higher than the activity found in patients with HEP with a G281E mutation.[dx.doi.org] Tetrahydro-8-chloro-3-methyl-5-phenyl-1H-3-benzazepin-7-ol SK&F-38393 2,3,4,5-Tetrahydro-7,8-dihydroxy-1-phenyl-1H-3-benzazepine D04 - POLYCYCLIC HYDROCARBONS D08 - ENZYMES, COENZYMES[decs.bvs.br] […] cutanea tarda, we used a human URO decarboxylase cDNA to analyze the organization and expression of the URO decarboxylase gene in lymphoblastoid cells from normal individuals[ncbi.nlm.nih.gov] The mother had reduced UROD activity (50% of normal), whereas the father had normal UROD activity.[ncbi.nlm.nih.gov]

    Missing: A glutaconyl
  • Glutaric Aciduria

    Disorders in tyrosine metabolism,チロシン代謝異常 1. tyrosinemia type I,チロシン血症Ⅰ型 98% 2. tyrosinemia type II,チロシン血症Ⅱ型 98% 3. tyrosinemia type III,チロシン血症Ⅲ型 98% 4. aromatic amino acid decarboxylase[jc-metabolomics.com] Both siblings have high residual glutaryl-CoA dehydrogenase activity, and are compound heterozygotes for two mutations - R227P and V400M reported to be disease-causing in[ncbi.nlm.nih.gov] Abstract Glutaric aciduria type II, also known as multiple acyl coenzyme A dehydrogenase deficiency, is an autosomal recessive, mitochondrial organic acid disorder that impairs[ncbi.nlm.nih.gov] Glutaconyl-CoA is the main toxic agent in glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). Med Hypotheses. 2005;65(2):330-3.[ghr.nlm.nih.gov] In the clinically active phase, conventional magnetic resonance imaging showed mild ventricular dilatation.[ncbi.nlm.nih.gov]

    Missing: A
  • Aromatic L-Amino Acid Decarboxylase Deficiency

    Simplified assays for human plasma L-dopa decarboxylase and liver L-dopa and 5-hydroxytryptophan decarboxylase, used to demonstrate the enzyme deficiency, are also reported[ncbi.nlm.nih.gov] The diagnosis was confirmed by the lack of AADC activity in plasma, and a point mutation in the AADC gene.[ncbi.nlm.nih.gov] This thesis also investigated the effect of altered availability of the AADC coenzyme pyridoxal 5‟-phosphate (PLP) on AADC activity, protein and expression.[discovery.ucl.ac.uk] L-lactate dehydrogenase deficiency Myoadenylate deaminase deficiency Pancreatic alpha-amylase deficiency Phosphoglycerokinase deficiency Specific enzyme deficiency Succinate-coenzyme[icd9data.com] Abstract This report presents the case of an adult male with aromatic L-amino acid decarboxylase deficiency who developed serious cardiac rhythm disturbances during treatment[ncbi.nlm.nih.gov]

    Missing: A glutaconyl
  • Acidaminococcus

    […] delta subunit (gcdD), glutaconyl-CoA decarboxylase gamma subunit (gcdC), and glutaconyl-CoA decarboxylase beta subunit (gcdB) genes, complete cds AF030576 3015 591001 * [[bacdive.dsmz.de] We developed a method for evaluating Cpf1 activity, based on target sequence composition in mammalian cells, in a high-throughput manner.[nature.com] BACKGROUND: Coenzyme A-transferases are a family of enzymes with a diverse substrate specificity and subunit composition.[ncbi.nlm.nih.gov] […] anaerobic bacteria グラム陰性嫌気性桿菌およびらせん菌 Gram-negative anaerobic straight, curved, and helical rod アシダミノコッカス属 Acidaminococcus 関連語: クロトニルCoA ( crotonyl-CoA ) ブチリル補酵素A ( butyryl-coenzyme[lsd.bioscinet.org] A biotin -dependent sodium pump: glutaconyl-CoA decarboxylase from Acidaminococcus fermentans [11].[wikigenes.org]

    Missing: A
  • Fusobacterium Nucleatum

    All the enzymes including a sodium ion pump glutaconyl-CoA decarboxylase are present in F. nucleatum ( 3 ).[dx.doi.org] Bacterial invasion was found to be necessary for F. nucleatum-induced nuclear factor kappa B (NF-κB) activation.[ncbi.nlm.nih.gov] Accordingly, increased NADPH oxidase activity and production of superoxide anion were observed in GFs after F. nucleatum infection, but not after P. gingivalis infection.[ncbi.nlm.nih.gov] Our data suggest that invasive Fn activates β-catenin signaling via a TLR4/P-PAK1/P-β-catenin S675 cascade in CRC.[ncbi.nlm.nih.gov] Coenzyme and cofactor metabolism.[dx.doi.org]

    Missing: A
  • Leigh's Disease

    […] of pyruvate carboxylase and alpha-ketoglutarate decarboxylase were within the normal limits.[ncbi.nlm.nih.gov] Cerebral pyruvate decarboxylase activity was also diminished. All enzyme activities were measured in total homogenates.[ncbi.nlm.nih.gov] An assay of muscle mitochondrial respiratory enzyme activities revealed a deficiency of NADH-coenzyme Q reductase.[ncbi.nlm.nih.gov] Similarly, there were no differences between the two sexes in transketolase activity, and no correlation between transketolase activity and age.[ncbi.nlm.nih.gov] Biochemical study finally revealed normal respiratory chain enzymes including NADH-coenzyme Q reductase, succinate coenzyme Q reductase and cytochrome c oxidase while other[ncbi.nlm.nih.gov]

    Missing: A glutaconyl
  • Levodopa

    Simplified assays for human plasma L-dopa decarboxylase and liver L-dopa and 5-hydroxytryptophan decarboxylase, used to demonstrate the enzyme deficiency, are also reported[ncbi.nlm.nih.gov] No significant group differences were found in brain activations to pure motor inhibition or error processing (stop success vs. error trials).[ncbi.nlm.nih.gov] Coenzyme Q10 is a supplement that has received a lot of attention in Parkinson’s.[medium.com] LD is extensively and rapidly metabolized by peripheral enzymes, namely, aromatic amino acid decarboxylase and catechol- O -methyltransferase (COMT).[ncbi.nlm.nih.gov] […] improvements in measures of activities of daily living, quality of life, and a specific subset of non-motor symptoms after treatment with LCIG.[ncbi.nlm.nih.gov]

    Missing: A glutaconyl
  • Cerebellar Ataxia

    Abstract Cerebellar ataxia associated with glutamic acid decarboxylase autoantibodies (GAD-ab) is a rare and usually slow progressive disease with moderate to severe gait[ncbi.nlm.nih.gov] Microglia are believed to be a long-term reservoir for HIV infection, causing persistent immune activation (ie, chronic inflammation).[ncbi.nlm.nih.gov] Abstract Primary coenzyme Q10 (CoQ 10 ; MIM# 607426) deficiencies are an emerging group of inherited mitochondrial disorders with heterogonous clinical phenotypes.[ncbi.nlm.nih.gov] Abstract Cerebellar ataxia is one of the most frequent syndromes associated with autoantibodies against glutamic acid decarboxylase (GAD-ab).[ncbi.nlm.nih.gov] After statin withdrawal, and treatment with coenzyme Q10 in some patients, progressive improvement of gait ataxia was observed.[ncbi.nlm.nih.gov]

    Missing: A glutaconyl
  • Thiamine

    […] elements in the presence (40 mg l(-1)) or absence of each tested antivitamins to estimate their influence on growth rate, enzyme activity and kinetic parameters of pyruvate decarboxylase[ncbi.nlm.nih.gov] BACKGROUND: Thiamine metabolites and activities of thiamine-dependent enzymes are impaired in Alzheimer's disease (AD).[ncbi.nlm.nih.gov] Thiamine diphosphate is a coenzyme of many enzymes, most of which occur in prokaryotes.[ncbi.nlm.nih.gov] The decarboxylase is composed of four subunits: E1-α, E1-β, E2 and E3. A specific kinase and phosphatase activate and deactivate, respectively, the enzyme complex.[ncbi.nlm.nih.gov] Also ThDP has non-coenzyme functions beyond its coenzyme role.[nature.com]

    Missing: A glutaconyl