protein metabolism 2 Flashcards
⮞ Melanin ⮞ Catecholamines ⮞ T3 ⮞ T4 ..are major derivatives of?
Phe & Tyr
This inborn error manifests:
⮞ mental retardation
⮞ mousy odor of urine
⮞ loss of NADPH/DHB [dehydroxybenzoic acid] (or both)
PKU
⮞ Ochronosis
⮞ arthritis
⮞ black urine
..is the manifestations of?
Alkaptonuria (triad of alkaptonuria) AKU
Inborn error with increase in Homogentisic Acid
Alkaptonuria (AKU)
The main reason why there is no melanin in Albinism
walang tyrosinase
A more serious type of Tyrosinemia, that causes renal and liver failure.
What is the enzyme deficiency?
⮞ Type 1 (hepatorenal tyrosinemia)
⮞ Fumarylacetoacetate hydrolase deficiency (FAH)
A less serious type of tyrosinemia, that causes eye and skin lesions together with mental retardation. name the deficiency din pls mwah
⮞Type 2. or (oculocutaneous tyrosenemia)
⮞ tyrosine aminotransferase deficiency (TyAT)
melatonin, serotonin and niacin are derivatives of?
tryptophan
What pathway converts Trp 🡆 niacin (B3)?
Kynerenine-anthranilate pathway (kyn-anth P)
2 vitamins needed to convert Trp 🡆 niacin?
B2
B6
This is due to B2 and B6 deficiency
pellagra
what are the 3 D’s of pellagra?
➟ Dermatitis
➟ Diarrhea
➟ Dementia
vitamin B6 deficiency would cause
xanthurenic acid formation
This Trp inborn error disease causes ; pellagra, cerebellar ataxia, intellectual deterioration. also what is the deficiency
Hartnup disease:
➟ Tryp oxygenase/ pyrollase deficiency.
➟ where Trp cannot convert to Niacin
⮞ accumulation of serotonin
⮞ 5-HIAA is found in the urine
Carcinoid syndrome
⮞ Impaired intestinal renal absorption of Trp (shows blue urine or indicanuria)
Blue diaper syndrome
What gets absorbed and gets converted to indican, that produces blue urine?
Indole
uronic acid is a keto acid from of
Histidine
For histidine 🡆 glutamic acid, it has to pass thru the formation of
FIGLU (N-formiminoglutamate)
FIGLU 🡆Glutamic acid thru
THFA (tetrahydrofolic acid) [vit B9]
what are the derivatives of Histidine?
⮞ Histidine
⮞ Carnosine
⮞ Anserine
⮞ convulsive seizures
⮞ has histidase deficiency
Histidenmia (no conversion of histidine 🡆 uronic acid)
a urocanase deficiency that has elevated excretion of uronic acid in the urine
Urocanic Aciduria
Branched amino acids are metabolized where?
⮞ Brain
⮞ Muscles
3 branched-chain amino acids
⮞ Valine
⮞ Leucine
⮞ Isoleucine
1st step in branched chain metabolism that produces keto acids
Transamination
Decarboxylation is the 2nd step in branched A.A metabolism, what is the product?
CoA
Deficiency in Maple syrup disease
a-ketoacid decarboxylase deficiency
In Maple Urine Disease there is mental retardation, whuay?
accumulations of Val, Leu, Ile in the brain (shii is toxic yo)
A cheesy odor of breath and body fluids
Isovaleric acidemia
Accumulation and deficiency in Isovaleric acidemia?
Accumulation:
⮞ Isovaleryl CoA
⮞ Deficiency: Isovaleryl CoA DH
2 substances derived from Lysine and use
saccharopine, pipecolicacid, used in industrial purposes
An accumulation of Lysine and deficiency in Saccharopine Dehydrogenase
HyperLYSinemia
Too much of this would inhibit Arginase of the urea cycle
Lysine
What non-essential amino acid can be acquired from threonine?
Glycine
In threonine degradation, conversion of a-ketobutyric acid is by
Threonine dehydratase
Major pathway for the catabolism of threonine proceeds through the
formation of glycine
What is needed to be done to yield aminoacetone?
dehydrogenation and decarboxylation
The catabolism of this protein proceed to the formation of cysteine
Methionine
In the catabolism of Methionine you first pass thru
SAM (S-Adenosyl-Methionine)
In the conversion of methionine to cysteine you pass thru the formation of
Homocysteine
SAM is a donor in what reactions?
Creatine, Choline, Epinephrine synthesis
⮞ Dangerous substance when it accumulates in the body
⮞ corrodes the lining of blood vessel wall
⮞ eventually develops to atherosclerosis
Homocysteine
What lowers Homocysteine levels?
Vitamin B6, B9 (folic acid), B12
What is the only B-complex stored in the liver, and too much of this can cause hepatic damage?
B12
An asymptomatic inborn error, that has an accumulation of Homocysteine. pls tell me the deficiency <3
⮞ Homocystinuria
⮞ deficient in Cystathionine Synthetase (CTS)
In Cystathioninuria, there is a deficiency in
Cystathionase
2 pathways for catabolism of Cysteine
⮞ Direct oxidative (Cysteine sulfinate pathway)
⮞ Transamination (3-Mercaptopyruvate pathway)
This can result in RENAL CALCULI, where there is defective transport of cysteine and basic amino acids
Cystinuria
Disease where Cystine accumulates in lysosomes, with a deficiency of what?
⮞ Cystinosis
⮞ Cystathionase deficiency
simplest amino acid <3
Glycine
In the degradation of glycine, what is the major route?
Glycine synthase (glycine cleavage system)
Glycine can be converted to what ‘in the major route’
⮞ NADH (reduced nicotinamide adenine dinucleotide)
⮞ CO2
⮞ NH3 (ammonia)
Glycine 🡆 serine and vice versa thru
Serine Hydroxymethyltransferase (SERHMT)
Oxidative deamination by glycine oxidase yields?
Glyoxylic acid
Uses of glycine
heme, purine, creatine synthesis. also conjugates with cholic acid to form bile salt, and benzoic acid to form hippuric acid
This causes the ff:
⮞ Diarrhea
⮞ Abdominal pain
⮞ Vomiting
Benzoic acid
5 AA that can be converted to KETOGLUTARATE:
🡆 K-HERQP The King - ketoglutarate is H - histidine E - glutamate/glutamic acid R - Arginine Queen's - Glutamine (Qt amine) Priority - Proline
Purely ketogenic means:
⮞ ONLY be converted to ketone bodies
⮞ NOT GLUCOSE
Leucine only.
BOTH ketogenic and glucogenic?
Phe, Tyr, Iso, Trypto, Lys
