Biochemistry week 15 Flashcards
Tryptophan metabolism results in (3)
- Niacin, serotonin , melatonin
Histidine metabolism results in
- Histamine
Glycine metabolism results in
- Heme
Arginine metabolism results in (3)
Creatine , urea , nitric oxide
Glutamate metabolism results in
GABA
What are the 20 amino acids broken down into
- Intermediates in carbohydrates and lipid metabloism
Name the amino acid that form ocaloacetate
- Aspartate through transamination
Name the amino acids that form a ketoglutarate via glutamate(4)
- Glutamine
-Proline
-Arginine
-Histidine
Name the amino acids that form pyruvate(5)
- Alanine, serine,Glycine,Cysteine, Threonine
Name the amino acids that form fumarate and the process
- Phenylalanine and Tyrosine
- PAH (phenylalanine hydroxylase ) catalyzes phenylalanine hydroxylation producing tyrosine
What type of amino acids are Phenylalanine and Tyrosine
- Glucogenic and ketogenix
What is PhenylKetonuria caused by
-Deficiency of phenylalanine hydroxylase activity due to:
-Defective enzyme
-Deficient BH4 cofactor
Name the three Elevated Phenylalanine metabolites
-Phenylpyruvate ,
-phenylacetate, phenyllactate
What are the effects of Phenylketonuria on the central nervous system
-Severe intellectual disability
-Developmental delay, -microcephaly,
-seizures
What are the effetcs of Phenylketonuria in relation to hypopigmentation
Leads to :
-Fair hair, light skin, blue eyes due to decreased melanin synthesis
What is used to detect elevated phenyalanine blood levels
- Laboratory testing
What is the function of the enzyme phenylalanaine hydroxylase
- converts phenylalanine to tyrosine
What is Albinism
- Genetic disorder affecting tryosine metabolism
What does Albinism lead to
- Mealanin deficiency, causing partial of full lack of pigment in skin, hair, eyes
What are the symptoms of Albinism
- Hypopigmentation of skin, hair and eyes
-Vison defects - Increased risk of skin cancer due lack of melanin production
What is Parkinsons disease caused by
-Dgeneration and depigmentation of neurons in the substantia nigra
What is the parkinson disease lead to
- Dopamine deficiency, affecting volutaary muscle movement
What is Methionine
Sulfur containing amino acid
What is the role of Methionine
It is converted to SAM, a major methyl donor
and also produces:
-Hcy(Homosysteine, which is linked to thrombosis
Name the steps in Methionine metabolsim
- SAM synthesis:
Methionine condenses with ATP to form SAM
-Methyl Transfer:
SAM donates methyl group forming SAH - SAH hydrolysis:
SAH is hydrolysed to Hcy and Adenosine
To regenerate methionine to maintain SAm what is reuqired
- Folic acid and vitamin B12
Name the amino acids that produce succinyl CoA
- Valine and isoleucine
- Threonine
Explain how valine and isoleucine produces succinyl CoA
- What is required
They are generated into propionyl CoA , then converted into methylmalonyl CoA and then to succinyl CoA
- Biotin and vitamin B12
How is threonine converted to succinyl CoA
-Dehydrated to a ketobutyrate , then converted itno propionyl then succinyl CoA
Name the 6 amino acids that form acetyl CoA or AcetoacetylCoA
- Tryptophan
-Leucine
-Isoleucine
-lysine
-Phenylalanine - Tyrosine
Name whether these amino acids are Ketogenic or Glucogenic:
-Tryptophan
-Leucine
-Isoleucine
-Lysine
-Tryptophan: Glucogenic , Ketogenic
-Leucine: Ketogenic
-Isoleucine: Ketogenic and glucogenic
-Lysine: Ketogenic
What amino acids are involved in Branched chain Amino Acid degredation:
- What does it require(5)
-What does it produce
-Isoleucine, Leucine,Valine
- Thiamine pyrophosphate, lipoic acid, FAD, NAD+ and CoA
- produce NADH
What are the end of the products of Catabolism
-Isoelcuine
-Valine
- Leucine
- Acetyl CoA and succinyl CoA
- Succinyl CoA
-Acetoacetate and Acteyl CoA
What is Maple syrup Urine disease
Autosomal recessive disorder
What is Maple Syrup Urine disease caused by
- Deficiency in branched -chain aketo acid dehydrogenase
What are the symptoms of Maple Syrup urine disease
- Male syrup like odor of urine
-Intellectual disability
How is maple syrup urine disease treated
- With a Synthetic formula free of BCAA( Branche chain a keto acid dehydrogenase
In relation to the maple syrup urine disease what is the symptoms of the accumaltion of the branched chain a keto acid dehyrogenase (BCAA) in the blood
- Vomitting
-Neuological problems
-Ketoacidosis
What is the active form of folic acid and where is it produced
-Tetrahydrofolate(THF)
-Produced from folate by dihydrofolate reductase in a 2 step reaction
What is the function of Tetrahydrofolate (THF)
Carries one-carbon units
Explain what happens in glutamine synthesis
- It converts glutamate to glutamine
How is serine formed
3-phosphoglycerate or from glycine via SHMT
What is glycine formed from
Serine via SHMT
What is cysteine formed from
-From Hcy (homocysteine) and serine, which is hydrolysed to cysteine and a ketobutyrate
What causes homocystinuria
Defect in cystathionine B -synthase
Give some characteristcs of homocystinuria
- Long limbs
-Lens dislocation
-Intellectual disbility
What does cystinuria cause
- Kidney stones
