Tay-Sachs

A rare and extremely severe genetic condition that affects the brain and nerves, most commonly found in babies who appear healthy at birth and seem to develop normally for the first few months of life. After this period, development slows and symptoms start. These babies lack an enzyme (protein) called Beta-hexosaminidase A (HEX A) which is necessary for breaking down fatty substance in the brain and nerve cells. There is no cure or effective treatment for this condition. Children affected will die usually around the age of 5. What condition could possibly be so fatal and devastating? Tay-Sachs.

This disease is distinguished by the onset of severe mental and developmental retardation starting around six months of age. Gradually, Tay-Sachs children lose motor skills and mental functions. The apparently healthy baby steadily stops smiling; crawling or turning over, loses its ability to grasp or reach out and, eventually, become blind, paralyzed and unresponsive to its surrounding. An early sign of the disease is the cheery-red spot in the retina of the eye examined only by the use of an ophthalmoscope. The association of the central nervous system develops quickly and affected children become totally incapacitated by two or five years of life.

Tay-Sachs is an inherited disease that only occurs when both parents transmit the defective gene to their child therefore this disease is recessive since it needs both malfunctioning genes to appear. Tay-Sachs disease is controlled by a pair of genes on chromosome 15 which code for the production of the enzyme Hex-A. Children with Tay-Sachs disease have no working version of this gene which usually breaks down excess fatty substance and without it; fats build up and eventually destroy the nerve cells of the affected child.

INTRODUCTION

The Twenties wan a decade which condidted of many well know events and

famous people. In which made the twenties part of out history today, such

events and people that made history in the twenties were Al Capone the well

mnow mafia leader from Chicago, Charlie Chaplain of the silent movies,

the annual Montreal Carninval in Canada, and the 60th anniversary of canada

in 1927. But these events and people are only a quarter of the history

help in the 1920's.

ENTERTAINMENT

Entertainment in the Twenties consisted of many famous people, it was

in the twenties when a man by the name of Walt "Elias" Disney (1901-66) a

cartoonest born in Chicago, in which he later graduated and later started

his own animation company which he introduced Disney's most popular ans

enduring cartoon characters "MICKEY MOUSE" in 1928. During the twenties

MICKEY entertained Canadians in such films as "Steam Boat Willie" (1928).

Other great cartoon films done be Disney include "Skeleton Dance" in

(1929).

The silent movies of the twenties is nothing to compaire with the

voiced movies we have today. The actors had to show a lot

of facial and

bady expressions to get the veiwers to understand what was happening in the

film. The most talented actor's in the twenties were Charlie Chaplai who

was known for his "tramp" look, Keaton with his stone facial expressions

(because he showed no emotion) and the famous romatic Radolph Valintino.

These three and many more entertained Canadians through the twenties.

In 1926 the voice era was coming in effect by Warner Brothers by the

Vitaphone. In 1927 Warner Brothers finally completed the first sound

picture called "The Jazz Singer"

To canadains evenings were not spent infront of t.v. Instead they would

listen to the radio. One for the radio stations listened to was CKCL.

They would listen to mostly music and live plays. Most of the music they

listened to was Jazz, and of course those were the days of the popular

ballroom dancing with the big bands. Other ways of entertainment were by

newspapers and books, One of the novels was "Strange Fugitives". Back in

the twenties Canadains authors produced 50 best sellins

Fashion

When the first world war