Histidinemia: a biochemical variant or a disease?

K, Virmani; Widhalm, Kurt

doi:10.1080/07315724.1993.10718291

Cited by 15 publications

(7 citation statements)

References 105 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…HAL is a key enzyme in histidine metabolism, and mutations that diminish HAL activity cause elevated histidine levels in vertebrates [35], [36]. To test the hypothesis that haly-1(lf) mutations cause elevated histidine, we developed methods to measure total histidine levels in C. elegans .…”

Section: Resultsmentioning

confidence: 99%

“…These results suggest that in humans with elevated levels of histidine, histidine can bind to zinc, and the complex can be excreted in the urine. The syndrome may predispose patients to disorders of the central nervous system [35], [36]. In a mouse model of histidinemia, the disease is autosomal recessive, and the histidine ammonia lyase gene located on chromosome 10 is predicted to encode a protein with a single amino acid change [45], [46], [47], [48].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Histidine Protects Against Zinc and Nickel Toxicity in Caenorhabditis elegans

et al. 2011

View full text Add to dashboard Cite

Zinc is an essential trace element involved in a wide range of biological processes and human diseases. Zinc excess is deleterious, and animals require mechanisms to protect against zinc toxicity. To identify genes that modulate zinc tolerance, we performed a forward genetic screen for Caenorhabditis elegans mutants that were resistant to zinc toxicity. Here we demonstrate that mutations of the C. elegans histidine ammonia lyase (haly-1) gene promote zinc tolerance. C. elegans haly-1 encodes a protein that is homologous to vertebrate HAL, an enzyme that converts histidine to urocanic acid. haly-1 mutant animals displayed elevated levels of histidine, indicating that C. elegans HALY-1 protein is an enzyme involved in histidine catabolism. These results suggest the model that elevated histidine chelates zinc and thereby reduces zinc toxicity. Supporting this hypothesis, we demonstrated that dietary histidine promotes zinc tolerance. Nickel is another metal that binds histidine with high affinity. We demonstrated that haly-1 mutant animals are resistant to nickel toxicity and dietary histidine promotes nickel tolerance in wild-type animals. These studies identify a novel role for haly-1 and histidine in zinc metabolism and may be relevant for other animals.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Histidine Protects Against Zinc and Nickel Toxicity in Caenorhabditis elegans

et al. 2011

View full text Add to dashboard Cite

show abstract

“…This transport system is unspecific with a K M value for histidine of approximately 30 µM (Bauza andLagunoff 1983, del Amo et al 2008). Once within the cells, two major pathways, depending on the tissue specific enzymes, are known for histidine degradation: either deamination to glutamate via urocanic acid, mainly in liver and skin by histidase and subsequently urocanase (Taylor et al 1991, Virmani andWidhalm 1993), or transamination in the liver to finally aspartate (Greenberg 1969). Additionally, histidine can be decarboxylated giving the biogenic amine histamine.…”

Section: Discussionmentioning

confidence: 99%

Histidine Metabolism After Bretschneider Cardioplegia in Cardiac Surgical Patients

Teloh

Ansorge²,

Petersen³

et al. 2018

Physiol Res

View full text Add to dashboard Cite

Bretschneider (histidine-tryptophan-ketoglutarate) solution with its high histidine concentration (198 mM) is one of many cardioplegic solutions, which are routinely used for cardiac arrest. The aim of this study was to evaluate the physiological biochemical degradation of administered histidine to histamine and its major urinary metabolite N-methylimidazole acetic acid. A total number of thirteen consecutive patients scheduled for elective isolated coronary artery bypass grafting with cardiopulmonary bypass were enrolled in the prospective observational designed study at the Department of Thoracic and Cardiovascular Surgery between 04/2016 and 06/2016. Patients received 1.7 l Bretschneider solution on average. Before and at the end of operation as well as in the postoperative course, urine samples gathered from the urinary catheter bag were analyzed. During the operative period, urinary histidine concentration significantly increased from 29 µmol/mmol creatinine to 9,609 µmol/mmol creatinine. Postoperatively, histidine excretion reduced while histamine as well as N-methylimidazole acetic acid excretion rose significantly. Patients showed elevated levels of histidine, histamine as well as N-methylimidazole acetic acid in urine, but no unmanageable hemodynamic instability possibly arising from the histamine’s biological properties. Chemically modified histidine might reduce uptake and metabolization while maintaining the advantages of buffer capacity.

show abstract

“…In subjects with this enzyme deficiency, histidine accumulates in the urine and plasma. Although not always present, mental retardation can occur (Virmani and Widhalm, 1993).…”

Section: Metabolic Defects In Essential Amino Acid Metabolismmentioning

confidence: 98%

A review of physiological and metabolic effects of essential amino acids

1998

View full text Add to dashboard Cite

The authors review ten essential amino acids with regard to their metabolic, physiologic and therapeutic effects throughout the human body. Physical properties of these biologically active compounds are discussed as a foundation for their diverse roles in special nitrogen containing products, neurotransmitters, and as alternative energy sources. Both normal and abnormal amino acid metabolism are considered in the areas of digestion, elimination of metabolic products, metabolic intermediates, and defects in these systems. Recent developments in therapeutic applications are further examined for clinical utility and as an economical alternative to traditional clinical treatment modalities.

show abstract

Histidinemia: a biochemical variant or a disease?

Cited by 15 publications

References 105 publications

Histidine Protects Against Zinc and Nickel Toxicity in Caenorhabditis elegans

Histidine Protects Against Zinc and Nickel Toxicity in Caenorhabditis elegans

Histidine Metabolism After Bretschneider Cardioplegia in Cardiac Surgical Patients

A review of physiological and metabolic effects of essential amino acids

Contact Info

Product

Resources

About