Week 4 Flashcards
(56 cards)
Peroxisomal FA oxidation
Long chain and branched chain fatty acid
Transfer electrons to O2, creates H2O2 –> Catalase
Reach 8 carbons –> carnitine
Lack of ETF or ETF:CoQ oxired
Glutaric acidemia
Mild respond to riboflavin
Primary carnitine deficiency
Lack of membrane transporter for carnitine
Weakness, cramping, hypoglycemia, fat accumulation
Secondary carnitine deficiency
CPT II deficiency
MCAD deficiency
Glu to Lys mutation
Hypoglycemia when glycogen is low, cant generate energy from fatty acid oxidation
Ketone bodies cant form
First cold
Zellweger sundrome
Lack of peroxisomes, accumulation of long chained and branched fatty acids
Refsum disease
Loss of alpha oxidation –> accumulation of phytanic acid
Myelin sheath
Adrenoleukodystrophy
ABCD1 gene mutation, peroxisome membrane transport protein
Lorenzo’s oil
ACOX1
Straight chain oxidase deficiency
ACOX2
Branched chain deficiency
Jamaican vomiting disease
Ackee fruit
Interferes with SCAD and MCAD
Severe hypoglycemia, increased fatty acids
Major minerals
Calcium, phopsphorus, potassium, sodium, chloride, magnesium
Trace minerals
Iron, zinc, copper, manganese, iodide, selenium, fluoride, molybdenum, chromium
Calcium absorption
Low levels: Active transport - vitamin D dependent
High levels: passive transport
Increase Ca absorption
Need (growth, pregnancy) and acidic medium
Reduced Ca absorption
Low vit D, low gastric acidity, high fiber, oxalates, phytates
PTH
Increases Ca absoprtion
Heme iron transport
No binding, easier entry
Non heme iron transport
DMT1, bind to apoferritin
Heme iron absorption
20-30%, unaffected by dietary components
Non heme iron absorption
2-10%, enhanced by Vit C, gastric acid, MFP factor
Reduced by phytates/oxalates, tannic acid
Zinc foods
Protein foods, legumes, nuts, whole grain
Zinc deficiency
Growth retardation, delayed sexual maturation, impaired immune system
A1AT accumulation
Emphysema in lung
Cell death and cirrhosis in liver
