Published 1975 | Version v1
Report Metadata-only

Investigation of 13C-valine metabolism in methylmalonic acidemia using nuclear magnetic resonance: identification of propionate as an obligate intermediate

Creators

  • 1. Yale Univ., New Haven

Description

[α-13C]- and [α,β-13C]valine were administered sequentially to a patient with methylmalonic acidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA

Additional details

Publishing Information

Imprint Title
Proceedings of the second international conference on stable isotopes
Imprint Pagination
p. 445-455.
Report number
CONF-751027--

Conference

Title
2. international conference on stable isotopes.
Dates
20 Oct 1975.
Place
Oak Brook, Illinois, USA.

INIS

Country of Publication
United States
Country of Input or Organization
United States
INIS RN
8315416
Subject category
S62: RADIOLOGY AND NUCLEAR MEDICINE;
Resource subtype / Literary indicator
Conference
Descriptors DEI
BIOCHEMICAL REACTION KINETICS; CARBON 13; DECARBOXYLATION; MALONIC ACID; METABOLIC DISEASES; METABOLISM; NUCLEAR MAGNETIC RESONANCE; PATIENTS; PROPIONIC ACID; SPECTROSCOPY; TRACER TECHNIQUES; VALINE
Descriptors DEC
AMINO ACIDS; CARBON ISOTOPES; CARBOXYLIC ACIDS; CHEMICAL REACTIONS; DICARBOXYLIC ACIDS; DISEASES; EVEN-ODD NUCLEI; ISOTOPE APPLICATIONS; ISOTOPES; KINETICS; LIGHT NUCLEI; MAGNETIC RESONANCE; MONOCARBOXYLIC ACIDS; NUCLEI; ORGANIC ACIDS; ORGANIC COMPOUNDS; REACTION KINETICS; RESONANCE; STABLE ISOTOPES