Sulphur Containing Amino Acids Overview

False (B)

Co-A or Taurine

<p>Methionine (D)</p> Signup and view all the answers

<p>False (B)</p> Signup and view all the answers

<p>Trimethyl glycine</p> Signup and view all the answers

<p>CBS = Classic homocysteinuria B6, B12 MTHFR = Non-classic homocysteinuria Dibasic amino acid transporter = Cystinuria CTNS gene = Cystinosis</p> Signup and view all the answers

<p>Atherosclerosis</p> Signup and view all the answers

<p>methionine</p> Signup and view all the answers

<p>Thromboembolism = Risk factor for atherosclerosis Ectopia lentis = Dislocation of lens Cyanide nitroprusside test = Magenta color indicated positive test Methionine restriction = Treatment for certain amino acid disorders</p> Signup and view all the answers

<p>Glutamic acid, Cysteine, Glycine (C)</p> Signup and view all the answers

<p>True (A)</p> Signup and view all the answers

<p>Arachnodactyly</p> Signup and view all the answers

<p>Homocysteine</p> Signup and view all the answers

<p>Cystinuria = An accumulation of cystine due to defective reabsorption. Arachnodactyly = Elongated fingers associated with classical homocysteinuria. Ectopia lentis = Displacement of the lens, common in certain syndromes. HMP shunt = Pathway that generates NADPH and ribose 5-phosphate.</p> Signup and view all the answers

<p>Energy production (D)</p> Signup and view all the answers

<p>False (B)</p> Signup and view all the answers

<p>Cyanide nitroprusside test</p> Signup and view all the answers

Sulfur-containing amino acids, with cysteine being a polar amino acid and methionine being a non-polar one.

A sulfur-containing amino acid derivative, a crucial intermediate in the conversion of methionine to cysteine and vice versa.

A molecule that transfers methyl groups in biochemical reactions. Crucial in converting homocysteine into methionine.

A therapy that involves providing supplemental cysteine, often for maintaining or improving health.

A dietary approach that limits the intake of methionine, often used to treat certain conditions.

Homocysteine is a crucial intermediary in the conversion of methionine to S-adenosylmethionine (SAM), the primary methyl donor in many biochemical processes.

The primary methyl donor in the body, essential for various metabolic reactions including DNA/RNA methylation, neurotransmitter synthesis, and lipid metabolism.

These are different types of hereditary disorders causing high homocysteine levels, leading to various health problems. Classic involves a defect in Cystathionine synthase, while non-classic may involve different enzyme deficiencies related to B vitamins.

A defect in a kidney transporter responsible for reabsorbing certain dibasic amino acids, resulting in their excretion in urine. These amino acids are Cystine, Ornithine, Lysine, and Arginine.

A lysosomal storage disorder caused by a malfunction in a lysosomal cystine transporter. This defect causes the accumulation of cystine inside the lysosomes leading to tissue damage.

Glutathione is involved in moving amino acids, using the Meister's cycle, primarily within the intestine, brain, and kidneys, requiring ATP.

Glutathione neutralizes harmful free radicals like H₂O₂ by reacting within a system that involves the HMP shunt.

Glutathione helps process foreign substances (xenobiotics) in the body, playing a crucial role in detoxification.

This condition is characterized by abnormally high levels of homocysteine in the blood and urine, leading to a decrease in cysteine synthesis.

Initially often asymptomatic; can cause skeletal deformities (like arachnodactyly, genu varum), developmental delays , and mental retardation, tall stature, high arched palate and others.

Deficiencies in Vitamins B6, Folate, or B12 can lead to elevated homocysteine levels, raising the risk of cardiovascular issues (CAD/CVA).

Folate (THFA) can become trapped in a form non-usable for metabolism, leading to functional folate deficiency.

Glutathione participates in biochemical reactions by serving as a coenzyme to catalyze reactions such as the isomerization of fumaroyl acetoacetate to malyl acetoacetate.