Related Experiment Video
Updated: Jul 11, 2026

Targeting Cysteine Thiols for in Vitro Site-specific Glycosylation of Recombinant Proteins
Published on: October 4, 2017
Cysteine: a conditionally essential amino acid in low-birth-weight preterm infants?
Maaike A Riedijk1, Ron H T van Beek, Gardi Voortman
1Department of Pediatrics, Division of Neonatology, Erasmus MC-Sophia Children's Hospital, University Medical Center, Rotterdam, the Netherlands.
Insights
Cystine is not essential for preterm infants born at 32-34 weeks gestation, as their bodies can synthesize enough. This finding suggests cystine is likely not conditionally essential for these infants when fed adequate methionine.
Area of Science:
- Nutritional biochemistry
- Neonatal physiology
- Amino acid metabolism
Background:
- Cystine, typically nonessential, may be conditionally essential in preterm infants due to immature synthesis pathways.
- Limited data exists on cystine requirements for low-birth-weight (LBW) preterm infants.
Purpose of the Study:
- To determine the cystine requirements in LBW infants (gestational age 32-34 weeks) one month after birth.
- To assess if cystine is conditionally essential in this population.
Main Methods:
- Utilized the indicator amino acid oxidation technique with [1-(13)C]phenylalanine.
- LBW infants received formulas with varying cystine concentrations (11-65 mg/100 mL) and ample methionine.
- Measured 13CO2 in breath to determine cystine requirement.
Main Results:
- Twenty-five LBW infants (gestational age 33±1 weeks) participated.
- No significant difference in phenylalanine oxidation was observed across different cystine intake groups.
- Determined cystine requirement to be less than 18 mg/kg/day.
Conclusions:
- Endogenous cystine synthesis appears sufficient in 4-week-old LBW preterm infants (32-34 weeks gestation).
- Cystine is likely not conditionally essential for enterally fed LBW preterm infants born at 32-34 weeks gestation, given adequate methionine supply.
- Established a cystine requirement threshold below 18 mg/kg/day for this infant population.
Background:
Cyst(e)ine can be synthesized de novo from methionine and serine and is, therefore, a nonessential amino acid in human adults. Several studies have suggested that cyst(e)ine might be a conditionally essential amino acid in preterm infants because of biochemical immaturity. No data are available on cyst(e)ine requirements in low-birth-weight (LBW) preterm infants.
Objective:
The aim was to determine cyst(e)ine requirements in LBW infants with gestational ages from 32 to 34 wk, measured 1 mo after birth with the use of the indicator amino acid oxidation technique.
Design:
LBW infants were randomly assigned to 1 or 2 of the 5 formulas containing graded cystine concentrations (11, 22, 32, 43, or 65 mg cyst(e)ine/100 mL) and generous amounts of methionine. After 24-h adaptation, cyst(e)ine requirement was determined by (13)CO(2) release from [1-(13)C]phenylalanine in expired breath. (13)CO(2) enrichment was measured by isotopic ratio mass spectrometry.
Results:
Cyst(e)ine requirement was determined in 25 LBW infants with a mean (+/-SD) gestational age of 33 +/- 1 wk and birth weight of 1.78 +/- 0.32 kg. Fractional oxidation of [1-(13)C]phenylalanine did not differ between the 5 groups.
Conclusions:
There is no evidence for limited endogenous cyst(e)ine synthesis in 4-wk-old LBW preterm infants born at gestational ages from 32 to 34 wk. It is safe to conclude that the cyst(e)ine requirement is <18 mg kg(-1) d(-1) providing generous amounts of methionine and that cyst(e)ine is probably not a conditionally essential amino acid in fully enterally fed LBW preterm infants born at 32-34 wk.
Related Concept Videos
Inborn Errors of Metabolism
Sulfur Assimilation
Amino Acid Biosynthetic Pathways
Overview of Protein Metabolism
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
Amino Acid Catabolism
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...

