SYSTEMIC DEFICIENCY OF THE 1ST COMPONENT OF THE PYRUVATE-DEHYDROGENASE COMPLEX

被引：69

作者：

KERR, DS

HO, L

BERLIN, CM

LANOUE, KF

TOWFIGHI, J

HOPPEL, CL

LUSK, MM

GONDEK, CM

PATEL, MS

机构：

[1] RAINBOW BABIES & CHILDRENS HOSP,DEPT BIOCHEM,CLEVELAND,OH 44106

[2] RAINBOW BABIES & CHILDRENS HOSP,DEPT MED,CLEVELAND,OH 44106

[3] CASE WESTERN RESERVE UNIV,VET ADM MED CTR,SCH MED,CLEVELAND,OH 44106

[4] PENN STATE UNIV,MILTON S HERSHEY MED CTR,DEPT PEDIAT,HERSHEY,PA 17033

[5] PENN STATE UNIV,MILTON S HERSHEY MED CTR,DEPT BIOCHEM,HERSHEY,PA 17033

[6] PENN STATE UNIV,MILTON S HERSHEY MED CTR,DEPT PATHOL,HERSHEY,PA 17033

来源：

PEDIATRIC RESEARCH | 1987年 / 22卷 / 03期

关键词：

D O I：

10.1203/00006450-198709000-00015

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

引用

页码：312 / 318

页数：7

共 42 条

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[6] ACTIVITIES OF BRANCHED-CHAIN 2-OXO ACID DEHYDROGENASE AND ITS COMPONENTS IN SKIN FIBROBLASTS FROM NORMAL AND "CLASSICAL-MAPLE-SYRUP-URINE-DISEASE SUBJECTS
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[7] NECROTIZING ENCEPHALOMYELOPATHY OF LEIGH - NEUROPATHOLOGICAL FINDINGS IN 8 CASES
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[8] COMPONENT-X - AN IMMUNOLOGICALLY DISTINCT POLYPEPTIDE ASSOCIATED WITH MAMMALIAN PYRUVATE-DEHYDROGENASE MULTI-ENZYME COMPLEX
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[J]. EUROPEAN JOURNAL OF BIOCHEMISTRY, 1985, 149 (03): : 641 - 648
[9] DEFECTIVE ACTIVATION OF THE PYRUVATE-DEHYDROGENASE COMPLEX IN SUB-ACUTE NECROTIZING ENCEPHALOMYELOPATHY (LEIGH DISEASE)
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[J]. ANNALS OF NEUROLOGY, 1979, 6 (06) : 483 - 494
[10] Estabrook R.W., 1967, METHOD ENZYMOL, V10, P41, DOI DOI 10.1016/0076-6879(67)10010-4

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