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Session 1 BC- AA deg

BioChem- PA TOURO 2011 BIOCHEM amino acid degredation

What is the cause of PKU deficiency in phenylalanine hydroxylase (PAH)
Why is PKU a problem PHE is elevated in tissues, plasma and urine it gets converted into phenylketones. High PHE decreases brain levels of other aromatic AA in brain
What are the symptoms of PKU mental retardation, failure to walk or talk, seizures, tremor, and microcephaly
How common is PKU allele in US and why is it not a problem in the womb 1 in 50, mother clears the Phe in the fetus. Because of this testing cannot not be done right after birth but should be done at second feeding
What is the treatment for PKU and how long should it continue low protein, low PHE diet that has to start 7-10 days after birth to avoid mental retardation. must follow diet for life
What is maple syrup urine disease recessive disorder deficiency of branched-chain a-ketoacid dehydrogenase (BCAKD) Accumulation of Leu, Ile, and Val and their corresponding a-keto acids in blood causes toxic effect on the brain
Symptoms of Maple syrup urine disease vomiting, dehydration, severe metabolic acidosis with no treatment disease leads to mental retardation, physical disabilities, and death
What are the types of maple syrup urine disease Classic type- no BCAKD activity severe acidosis in first week of life. Intermediate type- 4-5% of normal enzyme activity can be treated with large doses of thiamine.
What is the treatment of Maple syrup urine disease giving Leu, Ile, and Val in low doses in diet. need to be tested within first 24 hours and start treatment to continue normal development
What is Albinism it is deficiency in melanin production can be autosomal dominant, recessive,or x linked. Complete albinism is from deficiency of tyrosinase activity
What are the two types of melanine and their sub types Eumelanin (most abundant)- Black and Brown Pheomelanin has a pink to red hue.
What are mongolian spots birthmark results from entrapment of melanocytes in the dermis. normally a blue, black or brown mark.
What is Homocystinuria autosomal recessive disease with high plasma and urine levels of Met and homocysteine. Homozygous patients can have mental retardation and arterial disease
What is treatment for homocstinuria Low methionine diet with supplementation with vit B12 and B6 and folate which can help metabolize Met.
What is the major cause of death for people with homocystinuria about 25% die of myocardial infarction before age 30
What is Alkaptonuria black urine disease rare 1 in 1 million, they have high homogentisic acid, large joint arthritis, and black pigmentation of cartilage. Caused by deficiency in homogentisate oxidase
What is the treatment of Alkaptonuria no treatment late in life once you have the associated arthritis you just have to suffer. Can be prevented with nitisionone insectiside that inhibits homogentisic acid.
What are nonesential amino acids (definition not list) AA that can be synthesized by us
What are essential amino acids AA that can not be synthesized and we get them from the diet
Why are AA acids broken down? carbon skeletons give seven intermediate products used in sythesis of glucose and lipids. Production of energy by oxidizing them in the TCA cycle
Which amino acids are strictly ketogeneic Leucine, Lysine
Which AA can be both keto-genic and glycogenic Phenylalanine, Isoleucine, tyrosine, and Tryptophan.
AA that yield pyruvate or the intermediates of TCA cycle are called? Glycogenic or glucogenic the give rise to glucose or glycogen formation
AAA acids that yield acetoacetate or its precursors (acetyl or acetoaacetyl-CoA) are called what type of AA Ketogenic give rise to ketone bodies
Homocystein can be converted into two AA name them and the cofactor needed for the conversion Homocystein to Methionine needs Folate & Vit B12 Homocystein to Cystein needs Vit B6
What is the role of folic acid in AA metabolism It is a source of one carbon unit along with methanol, formaldehyde, formic acid and carbonic acid
What are the two carbon carriers in AA metabolism Tetrahydrofolic acid and S-adenosylmethionine(SAM)
What makes the active form of Folic Acid (THF) Dihydrofolate reductase
List the 9 essential amino acids His, Iso, Leu, Lys, Met, Phe, Thr, Trp, Val
Beans are a good source of which essential amino acds Lysine and Thr
Rice is a good source of which essential AA Met
What is Kwashiorkor childhood malnutrition due to insufficient protein uptake
How are the non essential AA made Made from intermediates of metabolism
Except Tyr, Phe, and Cys was is required for AA synthesis Sulfur from Met
Which nonessential AA is considered essential during childhood because the body cannot convert enough for growth Arg
What is transamination Transfer of amino group to alpha keto acids. Ala, Asp, Glut. each has their own transferase ie ALT
What is the only AA from glycolisis Ser
What are the consequences of defects in AA metabolism mental retardation and developmental abnormalities.
How are AA metabolism defects detected Through screening of new borns generally 10 to 20 markers are tested for abnormalities
Created by: smaxsmith
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