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Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.
Metab Brain Dis. 2002 Jun; 17(2):65-75.MB

Abstract

In this study we investigated the effects of the branched chain alpha-ketoacids accumulating in maple syrup urine disease (MSUD) on the concentrations of the high molecular weight neurofilament subunit (NF-H) associated with the cytoskeletal fraction of the cerebral cortex of 12-day-old rats. Cortical slices were incubated with alpha-ketoisocaproic acid (KIC), alpha-keto beta-methylvaleric acid (KMV) and alpha-ketoisovaleric acid (KIV) at concentrations ranging from 0.5 to 1.0 mM. The cytoskeletal fraction was extracted and the immunoreactivity for phosphorylated and total NF-H was analyzed by immunoblotting. The in vitro 32P incorporation into NF-H was also determined. Results showed that treatment of tissue slices induced with KMV increased Triton-insoluble phosphorylated NF-H immunoreactivity, with no alteration in total NF-H immunoreactivity. Furthermore, KIC treatment drastically increased the total amount of NF-H, whereas KIV did not change either phosphorylated or total NF-H immunoreactivity. KMV also increased the in vitro 32P incorporation into NF-H, confirming the highly phosphorylated NF-H levels detected in the immunoblot. These findings demonstrate that KIC and KMV alter the dynamic regulation of NF-H assembly in the cytoskeletal fraction. Therefore we may suggest that cytoskeletal disorganization may be one of the factors associated with the neurodegeneration characteristic of MSUD disease.

Authors+Show Affiliations

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brasil. rpureur@vortex.ufrgs.brNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12083338

Citation

Pessoa-Pureur, R, et al. "Effect of the Branched-chain Alpha-ketoacids Accumulating in Maple Syrup Urine Disease On the High Molecular Weight Neurofilament Subunit (NF-H) in Rat Cerebral Cortex." Metabolic Brain Disease, vol. 17, no. 2, 2002, pp. 65-75.
Pessoa-Pureur R, Funchal C, de Lima Pelaez P, et al. Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex. Metab Brain Dis. 2002;17(2):65-75.
Pessoa-Pureur, R., Funchal, C., de Lima Pelaez, P., Vivian, L., Oliveira Loureiro, S., de Freitas Miranda, R., & Wajner, M. (2002). Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex. Metabolic Brain Disease, 17(2), 65-75.
Pessoa-Pureur R, et al. Effect of the Branched-chain Alpha-ketoacids Accumulating in Maple Syrup Urine Disease On the High Molecular Weight Neurofilament Subunit (NF-H) in Rat Cerebral Cortex. Metab Brain Dis. 2002;17(2):65-75. PubMed PMID: 12083338.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex. AU - Pessoa-Pureur,R, AU - Funchal,C, AU - de Lima Pelaez,P, AU - Vivian,L, AU - Oliveira Loureiro,S, AU - de Freitas Miranda,R, AU - Wajner,M, PY - 2002/6/27/pubmed PY - 2002/12/19/medline PY - 2002/6/27/entrez SP - 65 EP - 75 JF - Metabolic brain disease JO - Metab Brain Dis VL - 17 IS - 2 N2 - In this study we investigated the effects of the branched chain alpha-ketoacids accumulating in maple syrup urine disease (MSUD) on the concentrations of the high molecular weight neurofilament subunit (NF-H) associated with the cytoskeletal fraction of the cerebral cortex of 12-day-old rats. Cortical slices were incubated with alpha-ketoisocaproic acid (KIC), alpha-keto beta-methylvaleric acid (KMV) and alpha-ketoisovaleric acid (KIV) at concentrations ranging from 0.5 to 1.0 mM. The cytoskeletal fraction was extracted and the immunoreactivity for phosphorylated and total NF-H was analyzed by immunoblotting. The in vitro 32P incorporation into NF-H was also determined. Results showed that treatment of tissue slices induced with KMV increased Triton-insoluble phosphorylated NF-H immunoreactivity, with no alteration in total NF-H immunoreactivity. Furthermore, KIC treatment drastically increased the total amount of NF-H, whereas KIV did not change either phosphorylated or total NF-H immunoreactivity. KMV also increased the in vitro 32P incorporation into NF-H, confirming the highly phosphorylated NF-H levels detected in the immunoblot. These findings demonstrate that KIC and KMV alter the dynamic regulation of NF-H assembly in the cytoskeletal fraction. Therefore we may suggest that cytoskeletal disorganization may be one of the factors associated with the neurodegeneration characteristic of MSUD disease. SN - 0885-7490 UR - https://www.unboundmedicine.com/medline/citation/12083338/Effect_of_the_branched_chain_alpha_ketoacids_accumulating_in_maple_syrup_urine_disease_on_the_high_molecular_weight_neurofilament_subunit__NF_H__in_rat_cerebral_cortex_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=12083338.ui DB - PRIME DP - Unbound Medicine ER -