Histidinemia.

Neurol India

Published: September 1974

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The essential amino acid histidine plays a central role in the manifestation of several metabolic processes, including protein synthesis, enzyme-catalysis, and key biomolecular interactions. However, excess accumulation of histidine causes histidinemia, which shows brain-related medical complications, and the molecular mechanism of such histidine-linked complications is largely unknown. Here, we show that histidine undergoes a self-assembly process, leading to the formation of amyloid-like cytotoxic and catalytically active nanofibers.

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Dual-emissive europium doped UiO-66-based ratiometric light-up biosensor for highly sensitive detection of histidinemia biomarker.

Anal Chim Acta

February 2024

Key Laboratory of Chemical Utilization of Plant Resources of Nanchang, College of Chemistry and Materials, College of Science, Jiangxi Agricultural University, Nanchang, 330045, China. Electronic address:

Background: Lanthanide metal-organic frameworks (Ln-MOFs) are a kind of emerging crystalline porous materials with high fluorescence and easy-to-tunable properties, making them ideal for sensing applications. However, current Ln-MOFs based fluorescent probes are primarily single-emissive or fluorescence-quenched, which greatly limited the detection performances such as sensitivity, accuracy and repeatability, thereby hindering their applications in efficient target monitoring and related disease diagnosis. To address these issues, the reasonable design of Ln-MOFs equipped with dual fluorescence emissions and light-up mode is urgently needed for a high-performance biosensor.

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The self-assembly of L-histidine might be the cause of histidinemia.

Sci Rep

October 2023

NMR Facility, Institute for Integrated Programmes and Research in Basic Sciences, Mahatma Gandhi University, Priyadarshini Hills P. O., Kottayam, 686560, Kerala, India.

L-Histidine is an essential amino acid with unique biochemical and physiological properties. Histidinemia is a disease condition caused by the elevated level of L-histidine in our blood. Mutations in the histidase, an enzyme for the breakdown of histidine, is the cause of the rise in histidine concentration.

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Histidinemia is a congenital metabolic disorder where the histidine (His) metabolism is blocked, resulting in increased concentrations of His in blood and urine. The disease causes an abnormal development of the patient's nervous system, which leads to many serious illnesses. Therefore, it is very important to diagnose early.

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A 25 μL analytical glass syringe has been used for isoelectric focusing (IEF) utilizing the stainless-steel needle and plunger as electrodes. The generation of protons and hydroxyl ions at the electrodes facilitated a neutralization reaction boundary (NRB) mechanism to focus different amphoteric compounds, such as hemoglobin, bovine serum albumin, R-phycoerythrin, and histidine, within minutes. After optimization of different experimental parameters affecting the IEF process and the coupling of the IEF syringe with electrospray ionization mass spectrometry (ESI-MS), a BGE composed of NHAc, 1.

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