PROGERIA

Progeria

Progeria

Introduction

The word Progeria has been derivative from a Greek word progeros means 'prematurely old'. In the Greek terms the word Pro refers to 'before' and the term 'geras' means 'old age'. This disorder is tremendously exceptional; it's a lethal genetic condition of a baby. Progeria is also called the Hutchinson-Gilford Progeria or HPG.HGPS is a genetic disorder.

The child, who is being suffered by the Progeria disorder, appeared as an old kid whose age increases exponentially because of this faster aging growth the kid of 6 years old appears as the old person of 70 years or so on, appearances are based on the condition of the particular kid. There are different sorts of the Progeria disorder the classic one is HGPS. Dr. Jonathan Hutchinson was firstly described this disorder in 1886 then Dr. Hastings Gilford was the second one who described this disorder a decade in 1897, both were described in England.

In Hayley's Page, it's recorded that up till now, there are 53 cases of this disorder have been known in entire world premises, and out of them 2 were seen in the UK. Progeria occurrence is reported that around 1 kid in every 4-8 million newborn babies. In this particular genetic disorder both girls and boy are equally risk having. Progeria is to be appeared in all sorts of races equally and during the last 15 years Progeria is being appeared in different sites of the different countries. And all are likewise suffering with this fatal disorder with no cure.(CNN, 2003)

Genetic Component

Kids who are being suffered by Hutchinson-Gilford Progeria Syndrome expire of brutal premature atherosclerosis at a typical age of 13 years. Even though the LMNA gene imperfection conscientious for this “premature aging syndrome” which has been acknowledged, biological mechanisms underlying the accelerated atherosclerosis are still not identified.( CNN, 2003)

The genetic derivation of most conditions of Hutchinson-Gilford Progeria Syndrome is a mutation from glycine GGC to glycine GGT in codon 608 of the lamin A (LMNA) gene. This triggers a cryptic splice donor location to make abnormal lamin A, and it disturbs the atomic membrane and adjusts transcription. Lamin are the major architectural constituents of a cage-like structure called the lamina (Hutchison, 2002). The lamina presents power and support for the inward atomic membrane (Hutchison, 2002). The mutant lamin protein merchandise is renowned as Progeria .Complex downstream body part scheme disturbance is the outcome of Progeria. Neither parent is a carrier of HGPS (MayoClinic.com). The gene change is a possibility incident influencing a lone egg or sperm just former to conception.

Metabolic Components

Recessive disorders are frequently emerged because of the deficiency in enzyme which show the way to metabolic abnormalities. The metabolic causes of Progeria are still a question but most of the research is being conducted.

Researchers founded that the secretion of Hyaluronic Acid in access causes these metabolic abnormalities. The mutation in Hyaluronic Acid (HA) metabolism can lie behind these diseases, conceivably because of invasive outcomes on angiogenesis.( Henig, 2005)

Signs

* Skin transforms analogous to that perceived in scleroderma (the ...