| Year |
Citation |
Score |
| 2007 |
Danner DJ, Barnes J, Mersey B. MicroRNA expression that controls the amount of branched chain α-ketoacid dehydrogenase in mitochondria of human cells Micrornas: From Basic Science to Disease Biology. 129-136. DOI: 10.1017/CBO9780511541766.012 |
0.336 |
|
| 2005 |
Mersey BD, Jin P, Danner DJ. Human microRNA (miR29b) expression controls the amount of branched chain alpha-ketoacid dehydrogenase complex in a cell. Human Molecular Genetics. 14: 3371-7. PMID 16203741 DOI: 10.1093/Hmg/Ddi368 |
0.452 |
|
| 2004 |
Muller EA, Danner DJ. Tissue-specific translation of murine branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region. The Journal of Biological Chemistry. 279: 44645-55. PMID 15302860 DOI: 10.1074/Jbc.M406550200 |
0.405 |
|
| 2004 |
Kasinski A, Doering CB, Danner DJ. Leucine toxicity in a neuronal cell model with inhibited branched chain amino acid catabolism. Brain Research. Molecular Brain Research. 122: 180-7. PMID 15010210 DOI: 10.1016/J.Molbrainres.2003.08.023 |
0.418 |
|
| 2003 |
Nellis MM, Kasinski A, Carlson M, Allen R, Schaefer AM, Schwartz EM, Danner DJ. Relationship of causative genetic mutations in maple syrup urine disease with their clinical expression. Molecular Genetics and Metabolism. 80: 189-95. PMID 14567968 DOI: 10.1016/S1096-7192(03)00144-6 |
0.649 |
|
| 2002 |
Nellis MM, Doering CB, Kasinski A, Danner DJ. Insulin increases branched-chain alpha-ketoacid dehydrogenase kinase expression in Clone 9 rat cells. American Journal of Physiology. Endocrinology and Metabolism. 283: E853-60. PMID 12217904 DOI: 10.1152/Ajpendo.00133.2002 |
0.647 |
|
| 2001 |
Nellis MM, Danner DJ. Gene preference in maple syrup urine disease. American Journal of Human Genetics. 68: 232-7. PMID 11112664 DOI: 10.1086/316950 |
0.661 |
|
| 2000 |
Doering CB, Williams IR, Danner DJ. Controlled overexpression of BCKD kinase expression: Metabolic engineering applied to BCAA metabolism in a mammalian system Metabolic Engineering. 2: 349-356. PMID 11120646 DOI: 10.1006/Mben.2000.0164 |
0.438 |
|
| 2000 |
Doering CB, Danner DJ. Amino acid deprivation induces translation of branched-chain α-ketoacid dehydrogenase kinase American Journal of Physiology - Cell Physiology. 279. PMID 11029306 DOI: 10.1152/Ajpcell.2000.279.5.C1587 |
0.469 |
|
| 2000 |
Doering CB, Danner DJ. Expression of murine branched-chain α-keto acid dehydrogenase kinase Methods in Enzymology. 324: 491-497. PMID 10989455 DOI: 10.1016/S0076-6879(00)24256-0 |
0.409 |
|
| 2000 |
Danner DJ. Mitochondrial import of mammalian branched-chain α-keto acid dehydrogenase complex subunits Methods in Enzymology. 324: 336-342. PMID 10989442 DOI: 10.1016/S0076-6879(00)24243-2 |
0.424 |
|
| 1998 |
Sitler TL, McKean MC, Peinemann F, Jackson E, Danner DJ. Import rate of the E1β subunit of human branched chain α-ketoacid dehydrogenase is a limiting factor in the amount of complex formed in the mitochondria Biochimica Et Biophysica Acta - Molecular Cell Research. 1404: 385-392. PMID 9739167 DOI: 10.1016/S0167-4889(98)00086-X |
0.423 |
|
| 1998 |
Doering CB, Coursey C, Spangler W, Danner DJ. Murine branched chain α-ketoacid dehydrogenase kinase; cDNA cloning, tissue distribution, and temporal expression during embryonic development Gene. 212: 213-219. PMID 9611264 DOI: 10.1016/S0378-1119(98)00182-6 |
0.438 |
|
| 1998 |
Danner DJ, Doering CB. Human mutations affecting branched chain alpha-ketoacid dehydrogenase Frontiers in Bioscience : a Journal and Virtual Library. 3. PMID 9609836 DOI: 10.2741/A299 |
0.479 |
|
| 1998 |
Danner DJ. Branched chain amino acid(BCAA) regulation of Branched chain α-Ketoacid Dehydrogenase(BCKD) Kinase Faseb Journal. 12. |
0.315 |
|
| 1997 |
McConnell BB, Burkholder B, Danner DJ. Two new mutations in the human E1 β subunit of branched chain α-ketoacid dehydrogenase associated with maple syrup urine disease Biochimica Et Biophysica Acta - Molecular Basis of Disease. 1361: 263-271. PMID 9375800 DOI: 10.1016/S0925-4439(97)00046-X |
0.423 |
|
| 1996 |
Lanterman MM, Dickinson JR, Danner DJ. Functional analysis in Saccharomyces cerevisiae of naturally occurring amino acid substitutions in human dihydrolipoamide dehydrogenase. Human Molecular Genetics. 5: 1643-8. PMID 8894701 DOI: 10.1093/Hmg/5.10.1643 |
0.423 |
|
| 1996 |
McConnell BB, McKean MC, Danner DJ. Influence of subunit transcript and protein levels on formation of a mitochondrial multienzyme complex Journal of Cellular Biochemistry. 61: 118-126. PMID 8726361 DOI: 10.1002/(Sici)1097-4644(19960401)61:1<118::Aid-Jcb13>3.0.Co;2-K |
0.428 |
|
| 1995 |
Leung PSC, Chuang DT, Wynn RM, Cha S, Danner DJ, Ansari A, Coppel RL, Gershwin ME. Autoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope Hepatology. 22: 505-513. PMID 7543435 DOI: 10.1016/0270-9139(95)90572-3 |
0.384 |
|
| 1993 |
Ellerine NP, Herring WJ, Elsas LJ, McKean MC, Klein PD, Danner DJ. Thiamin-responsive maple syrup urine disease in a patient antigenically missing dihydrolipoamide acyltransferase Biochemical Medicine and Metabolic Biology. 49: 363-374. PMID 8347380 DOI: 10.1006/Bmmb.1993.1037 |
0.429 |
|
| 1993 |
Turchany JM, Leung PS, Iwayama T, Jefferson DM, Ishida J, Yamaguchi M, Munoz S, Danner DJ, Dickson ER, Gershwin ME. Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis. Journal of Autoimmunity. 6: 459-66. PMID 8216688 DOI: 10.1006/Jaut.1993.1038 |
0.42 |
|
| 1992 |
Herring WJ, McKean M, Dracopoli N, Danner DJ. Branched chain acyltransferase absence due to an Alu-based genomic deletion allele and an exon skipping allele in a compound heterozygote proband expressing maple syrup urine disease Bba - Molecular Basis of Disease. 1138: 236-242. PMID 1547285 DOI: 10.1016/0925-4439(92)90043-M |
0.334 |
|
| 1992 |
McKean MC, Winkeler KA, Danner DJ. Nucleotide sequence of the 5′ end including the initiation codon of cDNA for the E1α subunit of the human branched chain α-ketoacid dehydrogenase complex Bba - Gene Structure and Expression. 1171: 109-112. PMID 1420356 DOI: 10.1016/0167-4781(92)90149-T |
0.36 |
|
| 1992 |
Lau KS, Chuang JL, Herring WJ, Danner DJ, Cox RP, Chuang DT. The complete cDNA sequence for dihydrolipoyl transacylase (E2) of human branched-chain alpha-keto acid dehydrogenase complex. Biochimica Et Biophysica Acta. 1132: 319-21. PMID 1420314 DOI: 10.1016/0167-4781(92)90169-Z |
0.337 |
|
| 1991 |
Herring WJ, Litwer S, Weber JL, Danner DJ. Molecular genetic basis of maple syrup urine disease in a family with two defective alleles for branched chain acyltransferase and localization of the gene to human chromosome I American Journal of Human Genetics. 48: 342-350. PMID 1990841 |
0.349 |
|
| 1990 |
Yoshida T, Bonkovsky H, Ansari A, Danner D. Antibodies against mitochondrial dehydrogenase complexes in primary biliary cirrhosis. Gastroenterology. 99: 187-194. PMID 2188870 DOI: 10.1016/0016-5085(90)91247-4 |
0.397 |
|
| 1989 |
Litwer S, Herring WJ, Danner DJ. Reversion of the maple syrup urine disease phenotype of impaired branched chain α-ketoacid dehydrogenase complex activity in fibroblasts from an affected child Journal of Biological Chemistry. 264: 14597-14600. PMID 2768232 |
0.404 |
|
| 1989 |
Van de Water J, Cooper A, Surh CD, Coppel R, Danner D, Ansari A, Dickson R, Gershwin ME. Detection of autoantibodies to recombinant mitochondrial proteins in patients with primary biliary cirrhosis. The New England Journal of Medicine. 320: 1377-80. PMID 2716784 DOI: 10.1056/Nejm198905253202104 |
0.385 |
|
| 1989 |
Danner DJ, Litwer S, Herring WJ, Elsas LJ. Molecular genetic basis for inherited human disorders of branched-chain α-keto acid dehydrogenase complex Annals of the New York Academy of Sciences. 573: 369-377. PMID 2699404 DOI: 10.1111/J.1749-6632.1989.Tb15012.X |
0.39 |
|
| 1989 |
Surh CD, Roche TE, Danner DJ, Ansari A, Coppel RL, Prindiville T, Rolland Dickson E, Gershwin ME. Antimitochondrial autoantibodies in primary biliary cirrhosis recognize cross-reactive epitope(s) on protein X and dihydrolipoamide acetyltransferase of pyruvate dehydrogenase complex Hepatology. 10: 127-133. PMID 2473022 DOI: 10.1002/Hep.1840100202 |
0.321 |
|
| 1989 |
DAVIS PA, FREGEAU DR, VAN DE WATER J, SURH C, ANSARI A, COPPELL R, DANNER D, GERSHWIN ME. Identification of the Acyltransferase (E2) Components of Branched-Chain ?-Keto Acid Dehydrogenase and Pyruvate Dehydrogenase Complexes as Autoantigens in Primary Biliary Cirrhosis Annals of the New York Academy of Sciences. 573: 441-443. DOI: 10.1111/J.1749-6632.1989.Tb15033.X |
0.414 |
|
| 1988 |
Danner DJ, Heffelfinger SC. Isolation of branched-chain α-keto acid dehydrogenase as active complex from bovine liver Methods in Enzymology. 166: 298-302. PMID 3071712 DOI: 10.1016/S0076-6879(88)66040-X |
0.414 |
|
| 1988 |
Danner DJ, Elsas LJ, Litwer S. Antibodies against branched-chain α-keto acid dehydrogenase proteins for use in defining human mutations and gene isolation Methods in Enzymology. 166: 108-114. PMID 3071695 DOI: 10.1016/S0076-6879(88)66016-2 |
0.426 |
|
| 1985 |
Danner DJ, Armstrong N, Heffelfinger SC, Sewell ET, Priest JH, Elsas LJ. Absence of branched chain acyl-transferase as a cause of maple syrup urine disease Journal of Clinical Investigation. 75: 858-860. PMID 3980729 |
0.375 |
|
| 1985 |
Fernhoff PM, Lubitz D, Danner DJ, Dembure PP, Schwartz HP, Hillman R, Bier DM, Elsas LJ. Thiamine response in maple syrup urine disease Pediatric Research. 19: 1011-1016. PMID 3903643 DOI: 10.1203/00006450-198510000-00012 |
0.394 |
|
| 1985 |
Litwer S, Danner DJ. Identification of a cDNA clone in λgt11 for the transacylase component of branched chain ketoacid dehydrogenase Biochemical and Biophysical Research Communications. 131: 961-967. PMID 2932110 DOI: 10.1016/0006-291X(85)91333-6 |
0.396 |
|
| 1984 |
Heffelfinger SC, Sewell ET, Elsas LJ, Danner DJ. Direct physical evidence for stabilization of branched-chain α-ketoacid dehydrogenase by thiamin pyrophosphate American Journal of Human Genetics. 36: 802-807. PMID 6475955 |
0.301 |
|
| 1983 |
Danner DJ, Priest JH. Branched-chain ketoacid dehydrogenase activity and growth of normal and mutant human fibroblasts: The effect of branched-chain amino acid concentration in culture medium Biochemical Genetics. 21: 895-905. PMID 6661177 DOI: 10.1007/Bf00483948 |
0.35 |
|
| 1983 |
Heffelfinger SC, Sewell ET, Danner DJ. Identification of specific subunits of highly purified bovine liver branched-chain ketoacid dehydrogenase Biochemistry. 22: 5519-5522. PMID 6652074 |
0.341 |
|
| 1981 |
Elsas LJ, Lubitz DS, Fernhoff PM, Dembure PP, Danner DJ. 713 THIAMINE RESPONSIVE MAPLE SYRUP URINE DISEASE Pediatric Research. 15: 561-561. DOI: 10.1203/00006450-198104001-00736 |
0.419 |
|
| 1980 |
Danner DJ, Lemmon SK, Elsas LJ. Stabilization of mammalian liver branched-chain α-ketoacid dehydrogenase by thiamin pyrophosphate Archives of Biochemistry and Biophysics. 202: 23-28. PMID 7396533 DOI: 10.1016/0003-9861(80)90401-4 |
0.373 |
|
| 1978 |
Danner DJ, Wheeler FB, Lemmon SK, Elsas LJ. In vivo and in vitro response of human branched chain α-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate Pediatric Research. 12: 235-238. PMID 643394 DOI: 10.1203/00006450-197803000-00016 |
0.42 |
|
| 1978 |
Danner DJ, Lemmon SK, Elsas LJ. Substrate specificity and stabilization by thiamine pyrophosphate of rat liver branched chain α-ketoacid dehydrogenase Biochemical Medicine. 19: 27-38. PMID 203270 DOI: 10.1016/0006-2944(78)90004-2 |
0.368 |
|
| 1978 |
Fernhoff PM, Danner DJ, Elsas LJ. 522 BIOCHEMICAL AND IMMUNOLOGIC EVIDENCE OF A LIPOAMIDE DEHYDROGENASE COMPONENT WITHIN THE BRANCHED CHAIN ALPHA-KETOACID DEHYDROGENASE COMPLEX Pediatric Research. 12: 450-450. DOI: 10.1203/00006450-197804001-00527 |
0.425 |
|
| 1975 |
Danner DJ, Elsas LJ. Subcellular distribution and cofactor function of human branched chain α-ketoacid dehydrogenase in normal and mutant cultured skin fibroblasts Biochemical Medicine. 13: 7-22. PMID 1167168 DOI: 10.1016/0006-2944(75)90135-0 |
0.388 |
|
| 1975 |
Danner DJ, Davidson ED, Elsas. LJ. Thiamine increases the specific activity of human liver branched chain α-ketoacid dehydrogenase Nature. 254: 529-530. PMID 1121328 DOI: 10.1038/254529A0 |
0.429 |
|
| 1974 |
Elsas LJ, Priest JH, Wheeler FB, Danner DJ, Pask BA. Maple Syrup Urine Disease: Coenzyme function and prenatal monitoring Metabolism. 23: 569-579. PMID 4857216 DOI: 10.1016/0026-0495(74)90085-7 |
0.433 |
|
| 1970 |
Connelly JL, Danner DJ, Bowden JA. [114] α-Ketoisocaproic acid or α-keto-β-methylvaleric acid dehydrogenase (beef liver) Methods in Enzymology. 17: 818-822. DOI: 10.1016/0076-6879(71)17289-8 |
0.356 |
|
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