Abstract

PHARMACOLOGICAL INTERVENTION AT HUNTINGTON'S DISEASE: A REVIEW STUDY

Amulya Jindal*, Ankit Lodhi, Deepak Tomar and Dhananjay Taumar

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Abstract

Huntington's chorea, also recognized as Huntington's disease (HGD), is a poisonous neurological illnesswhich affects three to seven persons per 100,000 of Eastern European heritage. HGD is thought to be one of the maximum frequent genetic abnormalities in the developed world. The condition often manifests itself between the ages of 30 and 50, is autosomal dominantly inherited, and is marked by chronic motor, cognitive, and mental symptoms. It causes gradual nerve cell destruction in the brain. As a result, the person's physical and mental capabilities might diminish normally within their prime active age, and symptomatic therapy is available. It is a hereditary condition that causes brain cell death.A Huntington gene mutation occurs in either of the two copies of a person's Huntington gene. The HGD gene was discovered in 1993. It is made up of a trinucleotide repeat, which is a repeating sequence of three base pairs. An excess of repeated CAG triplets in the gene leads in a protein with an unusually high amount of glutamine amino acids. On chromosome 4, a CAG triplet increases in the Huntington's gene.(4p 16.3), leading in an extensive polyglutamine stretch near the protein's N-terminus. Because it is a hereditary condition, there is a 50% risk that it will be passed down to the children.Currently, there is no cure for HGD, however clinical studies of powerful drugs are underway, and there are other promising prospective therapies in research. The estimation of important biomarkers for therapy assessment is currently under progress. Researchers prioritise therapeutic effectiveness over anything else. The current study's goal was to examine previous studies on pharmacological therapeutic intervention in HGD. A comprehensive literature search was conducted using Medline, The Cochrane Library's core database, and references lists in review papers as well as many different clinical reports.In this research, randomised controlled trials (RCTs)were categorised as Type I studies. Non-randomized, controlled clinical trials were used in type II investigations. Case reports were not included in type III studies, which instead assessed effectiveness, safety, and tolerability. HGD is a hereditary autosomal dominant condition with several subtypes based on its genetic background, diagnosis, symptoms, and therapy. The disorder is hereditary and can be discovered by testing for the HGD gene. Chorea symptoms include uncontrollable jerking and writhing motions. Although there is no cure for the abrupt development of chorea and behavioural problems, research is ongoing. Recent technological advances, on the other hand, have enabled scientists to discover and comprehend the gene responsible for this disease, providing tremendous promise for subsequent effective treatment.

Keywords: Huntington’s chorea, gene, mutant Huntington, toxicity.