BioChem Intel Deck I Fill in blanks Flashcards
What enzyme is responsible for leukotriene productions?
What is a Leukotriene?
Arachidonate 5-lipoxygenase
any of a group of biologically active compounds, originally isolated from leukocytes. They are metabolites of arachidonic acid, containing three conjugated double bonds.
The disease characterized by increased leukotriene synthesis is?
This disease cannot be treated with?
It should be treated with ?
Asthma
Cannot be treated with aspirin or (NSAIDs),
Inhibitors of 5-Lipoxygenase and antagonists of leukotriene receptors.
What enzyme is deficient in hereditary fructose intolerance?
Aldolase B
What metabolites accumulate and what are the symptoms of the above disease?
Aldolase B Deficency
Frutose 1-phosphate accumulates. ATP falls and AMP rises – hyperuricemia and lactic acidosis, hypoglycemia, impaired protein synthesis (f.e. decrease in blood clotting factors), impaired glycogenolysis. Diagnosis: fructose in urine.
You need Aldolase B to convert it into glyceraldehyde and dihydroxyacetonephosphate (DHAP).
Respiratory distress syndrome is seen most frequently in Preterm infants and it is caused by:
Insufficient production of Pulmonary Lung surfactant
Malignant carcinoid tumor is associated with increased synthesis of?
Vasoactive hormones (mainly serotonin)
What are the two biochemical functions of Thiamine?
The formation or degradation of Alpha-ketols by (Transketolase), and in the oxidative decarboxylation of alpha-keto acids (Pyruvate dehydrogenase complex, alpha-ketoglutarate dehydrogenase complex).
What is Nitrogen Balance?
Difference between consumed and excreted nitrogen
Where are HDLS formed and what is their function?
HDL particles are formed in the blood and within the liver and intestine. These high density Lipids are added to an apolipoprotein made by the liver and intestine and secreted into the blood.
- scavenge and removes bad cholesterol (LDLs) and send them to liver to be reporcessed or converted to bile to be secreted out of the system.
- maintains blood vessel walls by maintence in cleaning the surface in a anti athersclerosis prevention module.
Describe the Apolipoprotein Supply:
C-11 and APO E
They provide apolipoproteins Apo C-11 (activator of lipoprotein lipase) which is: a water-soluble enzyme that hydrolyzes triglycerides in lipoproteins, such as those found in chylomicrons and very low-density lipoproteins (VLDL), into two free fatty acids.
Apo E (required for endocytosis of lipoproteins) is a class of proteins involved in the metabolism of fats in the body.
remeber: Apoliprotein are proteins that bind lipids (oil-soluble substances such as fat and cholesterol) to form lipoproteins. They transport the lipids through the lymphatic and circulatory systems because we know the lipid components of lipoproteins are insoluble in water
What is the relationship of HDL’s and Reverse cholesterol Transport?
They transport cholesterol from peripheral tissues back to the liver (for bile acid synthesis or cholesterol disposal via the bile), and to steroidogenic cells for hormone synthesis.
HDL’s take up CE;s and unesterfied cholesterol from non heptatic tissues. The esterfication is catalyzed by LCAT (activated by HDL and Apo A-1). These esters are either returned to liver, or exchanged for triglycerides with VLDLs.
Faulty collagen synthesis is resulting from inherited defects causes several disease. List some that you learned during the course.
Ehlers-Danlos syndrome, Osteogenesis Imperfecta.
List 5 essential amino acids
Histidine, Leucine, Isoluciene, Methionone, Phelaline, Theronine, Tryptophan, and Valine
The predominantt nitrogen metabolite excreted in urine in humans is?
Urea
What protein serves as the primer for glycogen synthesis?
Fragments of glycogen or glycogenin (if glycogen is abscent)
Name the 3 major apolipoproteins and list their function
Apo A-1 major one of HDL, it activates LCAT
Apo B-100: VLDL, IDL, LDL is recognised by receptors for endocytosis
Apo A-4: is thought to act primarily in intestinal lipid absorption.
Apo C-II: activates lipoprotein lipase in capillaries (transferred from circulating HDLs to other lipoproteins)
Apo E: liver cells have these receptors.
Apo B types: form low-density lipoprotein LDL (sometimes referred to as “bad cholesterol”) particles. These proteins have mostly beta-sheet structure and associate with lipid droplets irreversibly. Most of the other apolipoproteins
(Apo -A) form high-density lipoprotein (“good cholesterol”) particles. These proteins consist of alpha-helices and associate with lipid droplets reversibly. During binding to the lipid particles these proteins change their three-dimensional structure. There are also intermediate-density lipoproteins formed by Apolipoprotein E.
In fasting, which molecule allosterically stimulates glycogen breakdown in the muscle?
AMP ( allosteric activator of glycogen phosphorylase )
The name of glycogen storage disease type 1 is?
The enzye deficient is?
What are the symptoms?
Von Gierke Disease
glucose-6-phosphatase
sever fasting hypoglycemia, fatty liver, progressive renal disease, growth retardation, delayed puberty, lactic acidemia, hyperlipidemia, hyperurecemia
What step follows glutamate formation during amino acid catabolism?
Oxidative glutamate deamination in which the amino group is liberated as free ammonia (catalysed by glutamate dehydrogenase)
Pellagra is a disease which results from a deficiency of?
Niacin, which has clinical features of dermatitis, diarrhea, dementia.
Statins are drugs used in?
to treat Hypercholesterolemia by inhibiting cholesterol synthesis. (reversible, competitve inhibitors of HMG CoA reductase)
What enzyme liberates arachidonic acid from the plasma membrane?
Phospholipase A2
Cellular cAMP levels can be increased by inhibiting what enzyme?
cAMP phosphodiesterase
What is glycogen storage disease type V?
Mc Ardle Syndrome
