The contents of this supplement can therefore account for all of this patient's abnormal findings on metabolic testing, the excess arginine being converted to ornithine
by arginase and [beta]-hydroxy-[beta]-methylbutyrate being synonymous with 3-hydroxyisovalerate (3-OH-IVA).
Successful early management of a female patient with a metabolic stroke due to ornithine
The age-related median values of seven amino acids (glycine, ornithine
, alanine, leucine/isoleucine, tyrosine, aspartic acid, and glutamic acid) drop sharply from the zero-time point collected shortly after birth to the median of the 6-12 month age group (Figure 1).
, and phenylalanine were three strongly and independently significant amino acids for the integrated metabolic panel and were finally adopted to develop the HOP panel based on the logistic regression model.
Abbreviations AMS: Altered mental status ICU: Intensive care unit OTC: Ornithine
transcarbamylase TPN: Total parenteral nutrition.
The OTC is a mitochondrial enzyme that catalyzes the reaction between carbamoyl phosphate and ornithine
to form citrulline, an essential step for the formation of nontoxic urea from ammonium .
marcescens produce magenta red pigment (prodigiosin) but former two species are negative for lysine and ornithine
On the other hand, one possible mechanism would be to depend upon elevated concentration of ornithine
, a precursor of polyamines, and might result from an increase in extra- hepatic arginase.
For in silico simulation of growth on arginine or succinate or ornithine
as the sole source of carbon and nitrogen, we set the flux rate of glucose and ammonium to zero and flux rate of desired metabolite's uptake/exchange reaction to 10 mmol x g [DW.sup.-1] x [hr.sup.-1].
This, however, appeared to be the case for arginase (i.e., enrichment of ornithine
derived from arginine) in solid tissues of living mice in the infusion model.
Putrescine is naturally produced in mammals by an enzyme called ornithine
decarboxylase,or ODC, and is easily absorbed and cleared by the body.
Urea Cycle Disorders (UCD) are an important group of inborn errors of metabolism resulting from deficiencies of six different enzymes inherited as autosomal recessive, with the exception of Ornithine
Transcarbamylase (OTC), which has X-linked inheritance.