THE LARGE SUBUNIT OF PORCINE INNER MITOCHONDRIAL MEMBRANE-BOUND BETA-OXIDATION COMPLEX IS A LONG-CHAIN ENOYL-COA HYDRATASE - 3-HYDROXYACYL-COA DEHYDROGENASE BIFUNCTIONAL ENZYME

被引:0
|
作者
YANG, SY [1 ]
机构
[1] NEW YORK STATE INST BASIC RES DEV DISABIL, STATEN ISL, NY 10314 USA
来源
FASEB JOURNAL | 1994年 / 8卷 / 07期
关键词
D O I
暂无
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
引用
收藏
页码:A1348 / A1348
页数:1
相关论文
共 46 条
  • [31] LONG-CHAIN 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY-THE MOST FREQUENT FATTY ACID OXIDATION DISORDER IN SELECTIVE SCREENING IN RUSSIA
    Baydakova, G., V
    Zakharova, E. Y.
    JOURNAL OF INHERITED METABOLIC DISEASE, 2010, 33 : S63 - S63
  • [32] Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review
    Hannah Fraser
    Julia Geppert
    Rebecca Johnson
    Samantha Johnson
    Martin Connock
    Aileen Clarke
    Sian Taylor-Phillips
    Chris Stinton
    Orphanet Journal of Rare Diseases, 14
  • [33] Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review
    Fraser, Hannah
    Geppert, Julia
    Johnson, Rebecca
    Johnson, Samantha
    Connock, Martin
    Clarke, Aileen
    Taylor-Phillips, Sian
    Stinton, Chris
    ORPHANET JOURNAL OF RARE DISEASES, 2019, 14 (01)
  • [34] Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria
    Tonin, Anelise M.
    Amaral, Alexandre U.
    Busanello, Estela N. B.
    Grings, Mateus
    Castilho, Roger F.
    Wajner, Moacir
    JOURNAL OF BIOENERGETICS AND BIOMEMBRANES, 2013, 45 (1-2) : 47 - 57
  • [35] Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria
    Anelise M. Tonin
    Alexandre U. Amaral
    Estela N. B. Busanello
    Mateus Grings
    Roger F. Castilho
    Moacir Wajner
    Journal of Bioenergetics and Biomembranes, 2013, 45 : 47 - 57
  • [36] CLONING AND EXPRESSION OF THE RAT-LIVER CDNA FOR PEROXISOMAL ENOYL-COA HYDRATASE, 3-HYDROXYACYL-COA DEHYDROGENASE IN LAMBDA-GT11 - TRANSCRIPTIONAL REGULATION OF ENZYME-ACTIVITY BY WY-14643 IN PRIMARY CULTURES OF RAT HEPATOCYTES
    CHATTERJEE, B
    MURTY, CVR
    OLSON, MJ
    ROY, AK
    EUROPEAN JOURNAL OF BIOCHEMISTRY, 1987, 166 (02): : 273 - 278
  • [37] AMINO-ACID-SEQUENCE SIMILARITIES OF THE MITOCHONDRIAL SHORT CHAIN DELTA-3,DELTA-2-ENOYL-COA ISOMERASE AND PEROXISOMAL MULTIFUNCTIONAL DELTA-3,DELTA-2-ENOYL-COA ISOMERASE, 2-ENOYL-COA HYDRATASE, 3-HYDROXYACYL-COA DEHYDROGENASE ENZYME IN RAT-LIVER - THE PROPOSED OCCURRENCE OF ISOMERIZATION AND HYDRATION IN THE SAME CATALYTIC DOMAIN OF THE MULTIFUNCTIONAL ENZYME
    PALOSAARI, PM
    VIHINEN, M
    MANTSALA, PI
    ALEXSON, SEH
    PIHLAJANIEMI, T
    HILTUNEN, JK
    JOURNAL OF BIOLOGICAL CHEMISTRY, 1991, 266 (17) : 10750 - 10753
  • [38] MOLECULAR-BASIS OF LONG-CHAIN 3-HYDROXYACYL-COA DEHYDROGENASE-DEFICIENCY - IDENTIFICATION OF THE MAJOR DISEASE-CAUSING MUTATION IN THE ALPHA-SUBUNIT OF THE MITOCHONDRIAL TRIFUNCTIONAL PROTEIN
    IJLST, L
    WANDERS, RJA
    USHIKUBO, S
    KAMIJO, T
    HASHIMOTO, T
    BIOCHIMICA ET BIOPHYSICA ACTA-LIPIDS AND LIPID METABOLISM, 1994, 1215 (03): : 347 - 350
  • [39] A common mutation of the long-chain hydroxyacyl-Co-A dehydrogenase enzyme of mitochondrial fatty acid beta-oxidation is not associated with non-alcoholic steatohepatitis
    Merriman, RB
    Bacon, BR
    Brunt, EM
    Tetri, BA
    Peters, MG
    Strauss, AW
    GASTROENTEROLOGY, 2001, 120 (05) : A543 - A543
  • [40] Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart - mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders
    Cecatto, Cristiane
    Hickmann, Fernanda H.
    Rodrigues, Marilia D. N.
    Amaral, Alexandre U.
    Wajner, Moacir
    FEBS JOURNAL, 2015, 282 (24) : 4714 - 4726