Literature DB >> 6464680

Abnormalities of muscle fibers in maple syrup urine disease.

G Ferrière, M de Castro, J Rodriguez.   

Abstract

Muscle fiber abnormalities are described in three cases of neonatal maple syrup disease. There were important variations in fiber diameters. Lesions consisted in focal or diffuse destruction of myofibrils. In view of recent biochemical and clinical data, a direct relation between elevated branched-chain amino acid levels and the muscular abnormalities seems improbable.

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Year:  1984        PMID： 6464680     DOI： 10.1007/bf00685251

Source DB:  PubMed          Journal:  Acta Neuropathol        ISSN： 0001-6322            Impact factor:   17.088


  19 in total

1.  RAPID EXAMINATION OF MUSCLE TISSUE. AN IMPROVED TRICHROME METHOD FOR FRESH-FROZEN BIOPSY SECTIONS.

Authors:  W K ENGEL; G G CUNNINGHAM
Journal:  Neurology       Date:  1963-11       Impact factor: 9.910

2.  Improved localization of phosphorylase by the use of polyvinyl pyrrolidone and high substrate concentration.

Authors:  O ERANKO; A PALKAMA
Journal:  J Histochem Cytochem       Date:  1961-09       Impact factor: 2.479

3.  Myofibrillar incorporation of 3H (G) L-leucine in pgrogressive muscular dystrophy and motor neuron disease.

Authors:  G Monckton; H Marusyk
Journal:  Neurology       Date:  1976-03       Impact factor: 9.910

4.  General stimulation of muscle protein synthesis by branched chain amino acids in vitro.

Authors:  M P Hedden; M G Buse
Journal:  Proc Soc Exp Biol Med       Date:  1979-04

5.  Congenital ophthalmoplegia, floppy baby syndrome, myopathy, and aminoaciduria. Report of a family.

Authors:  L J Hurwitz; N A Carson; I V Allen; J S Chopra
Journal:  J Neurol Neurosurg Psychiatry       Date:  1969-12       Impact factor: 10.154

Review 6.  Roles of branched-chain amino acids in metabolic regulation.

Authors:  S A Adibi
Journal:  J Lab Clin Med       Date:  1980-04

7.  Effect of L-carnitine on the oxidation of leucine and valine by rat skeletal muscle.

Authors:  V W Van Hinsbergh; J H Veerkamp; P J Engelen; W J Ghijsen
Journal:  Biochem Med       Date:  1978-08

8.  Branched-chain ketoacids reduce muscle protein degradation in Duchenne muscular dystrophy.

Authors:  P M Stewart; M Walser; D B Drachman
Journal:  Muscle Nerve       Date:  1982-03       Impact factor: 3.217

9.  Parenteral branched-chain amino acid treatment and avian dystrophy.

Authors:  M S Hudecki; C M Pollina; R R Heffner
Journal:  Muscle Nerve       Date:  1982 Jul-Aug       Impact factor: 3.217

10.  Improvements in epoxy resin embedding methods.

Authors:  J H LUFT
Journal:  J Biophys Biochem Cytol       Date:  1961-02
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  2 in total

1.  Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD.

Authors:  Davis S Sonnet; Monique N O'Leary; Mark A Gutierrez; Steven M Nguyen; Samiha Mateen; Yuehmei Hsu; Kylie P Mitchell; Antonio J Lopez; Jerry Vockley; Brian K Kennedy; Arvind Ramanathan
Journal:  Sci Rep       Date:  2016-07-04       Impact factor: 4.379

2.  Depletion of branched-chain aminotransferase 2 (BCAT2) enzyme impairs myoblast survival and myotube formation.

Authors:  Zameer N Dhanani; Gagandeep Mann; Olasunkanmi A J Adegoke
Journal:  Physiol Rep       Date:  2019-12
  2 in total

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