On the biomarkers and mechanisms of konzo, a distinct upper motor neuron disease associated with food (cassava) cyanogenic exposure

Roman M. Kassa, Nyamabo L. Kasensa, Victor H. Monterroso, Robert J. Kayton, John E. Klimek, Larry David, Kalala R. Lunganza, Kazadi T. Kayembe, Marina Bentivoglio, Sharon L. Juliano, Daniel Tshala-Katumbay

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Konzo is a self-limiting central motor-system disease associated with food dependency on cassava and low dietary intake of sulfur amino acids (SAA). Under conditions of SAA-deficiency, ingested cassava cyanogens yield metabolites that include thiocyanate and cyanate, a protein-carbamoylating agent. We studied the physical and biochemical modifications of rat serum and spinal cord proteins arising from intoxication of young adult rats with 50-200. mg/kg linamarin, or 200. mg/kg sodium cyanate (NaOCN), or vehicle (saline) and fed either a normal amino acid- or SAA-deficient diet for up to 2. weeks. Animals under SAA-deficient diet and treatment with linamarin or NaOCN developed hind limb tremors or motor weakness, respectively. LC/MS-MS analysis revealed differential albumin carbamoylation in animals treated with NaOCN, vs. linamarin/SAA-deficient diet, or vehicle. 2D-DIGE and MALDI-TOF/MS-MS analysis of the spinal cord proteome showed differential expression of proteins involved in oxidative mechanisms (e.g. peroxiredoxin 6), endocytic vesicular trafficking (e.g. dynamin 1), protein folding (e.g. protein disulfide isomerase), and maintenance of the cytoskeleton integrity (e.g. α-spectrin). Studies are needed to elucidate the role of the aformentioned modifications in the pathogenesis of cassava-associated motor-system disease.

Original languageEnglish (US)
Pages (from-to)571-578
Number of pages8
JournalFood and Chemical Toxicology
Volume49
Issue number3
DOIs
StatePublished - Mar 2011

Fingerprint

Sulfur Amino Acids
Manihot
Motor Neuron Disease
sulfur amino acids
Biomarkers
motor neurons
cassava
Neurons
biomarkers
Food
cyanates
Nutrition
Diet
spinal cord
Rats
Spinal Cord
Peroxiredoxin VI
Animals
Dynamin I
Two-Dimensional Difference Gel Electrophoresis

Keywords

  • Biomarkers
  • Cassava
  • Cyanate
  • Konzo
  • Linamarin
  • Proteomics

ASJC Scopus subject areas

  • Food Science
  • Toxicology

Cite this

On the biomarkers and mechanisms of konzo, a distinct upper motor neuron disease associated with food (cassava) cyanogenic exposure. / Kassa, Roman M.; Kasensa, Nyamabo L.; Monterroso, Victor H.; Kayton, Robert J.; Klimek, John E.; David, Larry; Lunganza, Kalala R.; Kayembe, Kazadi T.; Bentivoglio, Marina; Juliano, Sharon L.; Tshala-Katumbay, Daniel.

In: Food and Chemical Toxicology, Vol. 49, No. 3, 03.2011, p. 571-578.

Research output: Contribution to journalArticle

Kassa, RM, Kasensa, NL, Monterroso, VH, Kayton, RJ, Klimek, JE, David, L, Lunganza, KR, Kayembe, KT, Bentivoglio, M, Juliano, SL & Tshala-Katumbay, D 2011, 'On the biomarkers and mechanisms of konzo, a distinct upper motor neuron disease associated with food (cassava) cyanogenic exposure', Food and Chemical Toxicology, vol. 49, no. 3, pp. 571-578. https://doi.org/10.1016/j.fct.2010.05.080
Kassa, Roman M. ; Kasensa, Nyamabo L. ; Monterroso, Victor H. ; Kayton, Robert J. ; Klimek, John E. ; David, Larry ; Lunganza, Kalala R. ; Kayembe, Kazadi T. ; Bentivoglio, Marina ; Juliano, Sharon L. ; Tshala-Katumbay, Daniel. / On the biomarkers and mechanisms of konzo, a distinct upper motor neuron disease associated with food (cassava) cyanogenic exposure. In: Food and Chemical Toxicology. 2011 ; Vol. 49, No. 3. pp. 571-578.
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AU - Klimek, John E.

AU - David, Larry

AU - Lunganza, Kalala R.

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