Does lack of ceruloplasmin result in copper deficiency? yes/no? why?
No. b/c presence of other copper containing proteins that overt the symptoms
What are the consequences of ceruloplasmin deficiency?
Deficiency of iron metabolism. Accumulation of iron in the pancreas, retina and brain-> diabetes, blindness and neurological symptoms.
What form of iron can cross plasma membrane?
Where is the function of Cytochrome C?
Final component of ETC
What is the role of superoxide dismutase?
Key antioxidant enzyme vs. ROS
What is the role of tyrosinase?
key enzyme in synthesis of melanin
What are food sources for copper?
animal and plant orifin
What is the average copper intake in the US?
How common is copper deficiency?
Rare in the US
Name genetic disorders related to cupper metabolism
Wilson's disease and Menkel's syndrome
Describe Wilson's disease.
AR disease. Defect in Cu transporter required for biliary excretion (but not Cu uptake). Low serum ceruloplasmin, high urinary copper excretion and high hepatic copper content. Accumulation of Cu in liver, brain and kidney.
Describe Menkel's syndrome
Rare X linked disease. Defect in a distinct Cu transporter that transports Cu to the fetus or through the intestine after birth ->Cu deficiency. Low Cu levels in serum and most tissues. Very High Cu levels in intestine and kidney.
What drug is responsible for treating Wilson's disease?
What is the action of penicillamine?
chelating agent. Solubilizes copper and promotes its urinary excretion and dietary zinc, which inhibits copper absorption.
What are the symptoms in Wilson's disease?
Mental degeneration and liver damage
What are the symptoms in Menkel's syndrome?
Infancy: slow growth, mental degeneration (irreversible), kinky white hair. Most individuals die within the first few years of life
How to get adequate amount of vitamins and minerals in diet?
Vegetable and fruit
When do vitamin/mineral supplementation have significant health benefits?
If health conditions limit absorption, transport/metabolism of vitamins or minerals. Calcium for teens and young adults
What are essential amino acids?
Aa that cannot by synthesized by humans . Must be take by diet
What is Vitamin B12?
What is the role of Vitamin B12?
Enzymatic Cofactor for methylation reactions
Name the 9 essential aa .
His, Iso, Leu, Lys, Met, Phe, Thr, Trp, Val
What is the aa intake requirement for male adults?
What is the aa intake requirement for female adults?
What are semiessential aa?
Aa synthesized from essential aa
Name two semiessential aa
cys and tyr
Which aa are considered essential in infants?
Describe protein requirements in rapidly growing infants
high protein need, low endogeneous rate of cysteine synthesis. Cysteine is essential
Which aa are considered essential in pre-term infants?
tyrosine, glutamine, arginine, proline, and glycine
Which aa is essential during critical illness?
Name the essential FA
linoleic and linolenic acid
What is linoleic acid?
How many carbon atoms are found linoleic acid? How many double bonds?
Where are the double bonds found in linoleic acid?
9th and 12 th position according to Delta system
What is linolenic acid?
How many carbon atoms are found in linolenic acid? How many double bonds?
Where are the double bonds found in linoleic acid?
9th, 12th, and 15th position
What is the role of essential FA?
1. maintaining function and integrity of membranes 2. precursors for the synthesis of eicosanoids 3.Critical signaling molecules in inflammatory responses, blood clotting, etc..
Name 3 eicosanoids
prostaglandin, thromboxanes, leukotrienes
When do symptoms associated with essential FA deficiency manifest? Why>
Months to manifest b/c large endogenous stores of essential FA
What is the difference between omega and delta system?
Delta system counts carbon from the carboxyl end. Omega system counts from the methyl end. For omega, only the position of the double bond most proximal the methyl end is given. Any additional double bonds always separated by 3 carbons like for linoleic acid
What is the nomenclature for FA?
1st number is the total number of C atoms. the second number is the number of double bonds.
Why FA essentials? Name their characteristics
1. Humans lack the enzyme req. to insert a double bond 3 or 6 carbons from the omega end. 2. very low quantity is needed but required 3. deficiencies are rare in general population 4. Essential diet component for enteral/parenteral nutrition
What is the amount of essential FA required?
1 tbs of plant oil/d
What role do non essential dietary components play in our health?
Dietary supplement can influence positively or negatively individuals health. Can be produced by humans in sufficient quantities
Name non essential dietary components
Name semi essential nutrients.
choline, taurine, carnitene
What is a dietary fiber?
Plant-derived carbs and lignins that are not digested in human stomach or small intestine
Why are dietary fibers beneficial for our health?
Decrease intestinal transit time and increase ease of elimination of feces, reducing risk for development of diverticulitis, hemorrhoids, and colon cancer
What role do high fiber food play in our diet?
Aid weight control by increasing appetite satiety w/o yielding much energy and slowing carbohydrate absorption
Name dietary fibers
cellulose, pectins, gums, mucilages
What are the glycosidic bonds found in cellulose?
beta glycosidic bonds
What are insoluble fiber?
ingestible dietary fibers. Do not dissolve in water and add bulk to feces
What are soluble fibers?
Dissolved/swell in water. Digested by bacteria in large intestine into short chain FA and gases.
What are food sources for insoluble fiber?
wheat bran, wheat products, brown rice
What are food sources for soluble fibers?
fruits, carrots, oats, kidney beans
What is the energy contained in short chain FA?
3kcal/g if absorbed
What is cholesterol?
membrane structure. precursor to steroids, bile acids
What is lycopene?
caretenoid found in tomatoes , red fruits and vegetables
What are the health benefits of lycopene?
Anti-oxidant for free radicals. Reduce risk for cancers, cvd, osteoporosis, diabetes, macular degeneration. Not FDA approved
What are semi-essential nutrients?
Nutrients that can be synthesized by humans, but under certain circumstances could be limiting