proteins Flashcards
1
Q
kwashiorkor or marasmus
- <1 year old
- due to low calorie intake
- severe growth retardation
- severe reduction in body weight
- shrunken abdomen and subcutaneous fat not preserved
- prominent ribs
- voracious feeder
- no fatty liver
- oedema not present
- an old man like face
- no hair changes noted
- dry and wrinkled skin
- alert but irritable
- requires adequate amount of protein, fat, and carbohydrate
A
marasmus
2
Q
immunoglobulin
- (?) is the most abundant antibody in plasma and lymph
- (?) is the main antibody in mucous secretion
- (?) is the first antibody that appears in response to antigenic stimulation
- (?) is the antibody for allergic and anaphylactic reactions
- (?) is present mostly on the surface of B cells.
A
- IgG
- IgA
- IgM
- IgE
- IgD
3
Q
proteins
- Proteolytic fragments of collagen I formed during bone resorption
- CTX is a biochemical marker of bone resorption that can be detected in serum and urine
A
cross-linked c-telopeptides
4
Q
complement
second most abundant
A
C4
5
Q
CLINICAL SIGNIFICANCE
* decreased in hepatic damage, acute-phase inflammatory response, and tissue necrosis
- a low level is a sensitive marker of poor nutritional status
- increased in patients receiving steroids, in alcoholism, and in chronic renal failure
A
prealbumin
6
Q
- Single polypeptide chain that is a component of the light chain of HLA class I
- Freely filtered by the glomerulus and completely reabsorbed
- Increased: SLE, RA, Renal Failure, Multiple Myeloma, HIV
A
B2-microglobulin
7
Q
globulin
Most important function: inhibition of the protease neutrophil elastase Abnormal form of AAT can also accumulate in the liver and cause cirrhosis Major component of a1-globulin band → deficiency of AAT seen as lack of an a1-globulin band on SPE
A
alpha-1-antitrypsin
8
Q
globulin
- Natural defense and enhanced immune response by cell lysis
- Synthesize by the liver
A
complement
9
Q
globulin
- Synthesized by the developing embryo and fetus, thought to protect the fetus from immunologic atack by the mother
- No known function in normal adults
Clinical significance
* Elevated AFP. neural tube defects (e.g. spina bifda). presence of twins
- Low AFP: increased risk for Down syndrome (trisomy 21)
- Tumor marker for hepatocellular carcinoma, some testicular carcinomas
A
alpha-1-fetoprotein
10
Q
significant proteins
- It is a group of proteins consisting of α1, α2, β, and γ fractions.
- It is usually measured by subtracting the value of serum albumin from the total protein concentration.
- Method for measurement: Indirect method
A
globulin
11
Q
classification
- Contain peptide chains composed of amino acids linked by peptide bonds.
- These may be fibrous (fibrinogen, troponins, collagen) and globular (hemoglobin, plasma proteins, enzymes, peptide hormones) in shape
A
simple proteins
12
Q
proteins
- cysteine protease inhibitor
- New marker for changes in GFR
- Used for screening and monitoring kidney dysfunction
A
cystatin c
13
Q
alpha-2-macroglobulin
pitting edema
A
hypoalbuminemia
14
Q
globulin
- glycoproteins produced by plasma B cells
- Increased: Humoral response, Autoimmune disorder, Allergic reaction
- Decreased: Hypogammaglobulinemia,
Immune deficiency
A
immunoglobulins
15
Q
proteins
- Glycoprotein produced by fetal membranes responsible for the cellular adhesiveness of placenta and membranes to the decidua.
- Fetal (?) is produced at the boundary between the amniotic sac and the decidua (the lining of the uterus) and functions to maintain the adherence of the placenta to the uterus.
- Test for assessment of the risk for preterm delivery in women between 24 to 35 weeks gestational age
A
fibronectin
16
Q
alpha-2-macroglobulin
due to the loss of anti-thrombin III
A
hypercoagulate state
17
Q
globulin
- Autosomal recessive
- Decreased levels of ceruloplasmin
Excess storage of copper in various organs
* Liver → hepatic cirrhosis
- Brain → neurologic damage
- Cornea → Kayser-Fleischer rings
Other laboratory findings
* Total serum copper
- decreased Free serum copper increased
- Urinary copper increased
A
wilson’s disease
18
Q
alpha-2-macroglobulin
may result in fatty casts in the urine
A
hyperlipidemia and hypercholesterolemia
19
Q
globulin
- Transports lipids (HDL)
CLINICAL SIGNIFICANCE
* decreased positive risk factor for atherosclerosis and positive risk factor for Carbohydrates
A
alpha-1-lipoprotein
20
Q
formula for globulin
A
total protein - albumin
21
Q
globulin
- Copper-containing (contains >90% of total serum copper)
- Used in the diagnosis of Wilson’s disease
A
ceruloplasmin
22
Q
- Protein present in the highest concentration in serum
Functions:
* Provide nearly 80% of colloid osmotic pressure (COP) of intravascular fluid
- buffers pH
- binds to various substances in blood
- Negative acute-phase reactant
A
albumin
23
Q
protein
- Insoluble fibrous protein aggregates form due to alteration in the secondary structure known as B-pleated sheets
- Low (?) and High Tau protein: suggestive of Alzheimer’s Disease
- Abnormal (?) and Tau: Dementia
A
amyloid
24
Q
classification
- These comprise a protein (apoprotein) and a nonprotein moiety (prosthetic group).
Examples:
Metalloprotein
Lipoproteins
* Lipid attached
Mucoproteins or proteoglycans
* Mucin (higher CHO content than protein)
Glycoproteins
* proteins with 10%-40% carbohydrate content
Nucleoproteins
* chromatin (combined with nucleic acids)
A
conjugated proteins
25
# globulin
* Transports lipids (LDL)
* Increased: Hyperlipoproteinemia, atherosclerosis, CVD
B-lipoprotein
26
A disease marked by severe protein malnutrition and bilateral extremity swelling.
kwashiorkor
27
# synthesis
DNA to RNA to PROTEIN
central dogma
28
# levels/structure
number and types of amino acids in the specific amino acid sequence
primary
29
# globulin
* Large protein that inhibits proteases such as trypsin thrombin, kallikrein, and plasmin
* Increased In nephrotic syndrome (large size aids in Its retentlon)
* a major component of the α2 -globulin fraction, is a tetramer of four identical subunits synthesized by the liver
alpha-2-macroglubilin
30
# significant proteins
* Aka Transthyretin
* Migrates before albumin in the serum electrophoresis
* Fxn: Transport protein for thyroid hormones; transports vitamin A by forming a complex with retinol-binding protein
prealbumin
31
# globulin
* Inhibits serine proteases
CLINICAL SIGNIFICANCE
* increased in inflammation
inter-a-trypsin inhibitor
32
# globulin
* Produced by hepatocytes
* Major carrier protein of Vitamin D
* Transports Vitamin D and bind actin
* Important in bone formation and immune system.
CLINICAL SIGNIFICANCE
* increased: 3rd trimester of pregnancy intake of estrogen oral contraceptives
* decreased: liver disease protein-losing syndrome
GC-globulin / group specific component (Gc)
33
# globulin
* Glomerular disorder characterized by proteinuria (>3.5 g/day)
* Pathophysiology: Disruption of the electrical charges that produce the tightly fitting podocyte barrier resulting in massive loss of protein and lipids.
nephrotic syndrome
34
# globulin
* precursor of a fibrin clot, elevate with other acute phase reactants
* one of the largest proteins in plasma
* classified as a glycoprotein
* normally seen between beta-gamma region in electrophoresis
CLINICAL SIGNIFICANCE
* INCREASED: Inflammation, Pregnancy, Use of oral contraceptives
* DECREASED: Decreased during extensive coagulation
fibrinogen
35
# proteins
* AKA: Prostaglandin D synthase
* Marker for CSF leakage
* Potential marker in detecting impaired renal function but less sensitive than Cystatin C.
B-trace protein
36
# proteins
* There is an inverse correlation between body mass index and (?) values
Lower levels correlate with:
* Increased risk of heart disease
* Type 2 Diabetes
* Metabolic syndrome
* Obesity
adiponectin
37
# pathway
degrades extracellular proteins
lysosomal pathway
38
# levels/structure
It is the association of two or more polypeptide chains to form a functional protein molecule
quarternary
39
reference range for globulin
2.3 - 3.5 g/dl
40
# globulin
* One of the COPDs (chronic obstructive pulmonary diseases )
* **Most common cause**: smoking
* **Pathophysiology**: excessive inflammation or lack of AAT leads to destruction of alveolar air sacs → loss of elastic recoil and collapse of aiways during exhalation → obstruction and air trapping
* Dyspnea, cough with minimal sputum
* Pink puffers, "barrel-chest", hypoxemia
emphysema
41
Severe manifestation of protein-energy malnutrition (PEM).
marasmus
42
TWO MAIN ROUTES FOR CONVERTING INTRACELLULAR PROTEINS TO FREE AMINO ACIDS
* Lysosomal pathway
* Cytosolic pathway
43
# alpha-2-macroglobulin
increased risk of infection
hypogammaglobulinemia
44
# globulin
* Transports lipids (VLDL)
* Increased: Hyperlipoproteinemia, atherosclerosis
* Decreased: Starvation
pre-B-lipoprotein
45
# globulin
* Serine protease inhibitor
* Binds and inactivates Pancreatic Elastase, Mast cell, Chymase, and Chymotrypsin.
* Associated with the pathogenesis of Alzheimer’s disease because it is an integral component of the amyloid deposits
alpha-1-antichymotrypsin
46
# pathway
degrades intracellular proteins
cytosolic pathway
47
# globulin
* AKA: Orosomucoid
* A major plasma glycoprotein that is negatively charged
* Produced by the liver
* An acute phase reactant
* Useful diagnostic tool in neonatal bacterial infection (NOT WIDELY USED)
CLINICAL SIGNIFICANCE
* increased: Pregnancy, Cancer, Pneumonia, RA, Cell proliferation, stress and surgery
* decreased: Nephrotic syndrome
alpha-1-acid glycoprotein
48
# globulin
* Transports two molecules of ferric iron
* Negative acutephase reactant
* Major component of the beta-globulin fraction
* Tested to determine cause of anemia (e.g. increased levels in IDA)
transferrin
49
CLINICAL SIGNIFICANCE
* **decreased**: liver disease, malnutrition, malabsorption, kidney loss and hemodilution
* **increased**: dehydration
albumin
50
# levels/structure
regularly repeating structures stabilized by hydrogen bonds between the amino acids within the protein (e.g. a-helix, B pleated sheet, turns)
secondary
51
# kwashiorkor or marasmus
* 1-5 years old
* due to low protein intake
* mild growth retardation
* mild reduction in body weight
* protruding abdomen and subcutaneous fat reserved
* ribs not very prominent
* poor appetite
* enlarged fatty liver
* oedema present
* moonfacies
* sparse hair
* flaki paint-like skin
* lethargic
* requires adequate amount of protein
kwashiorkor
52
# globulin
* Precipitates with C substance, a polysaccharide of pneumococci
* Functions in opsonization
* One of the first acute-phase proteins to rise in inflammatory disease
* High or increasing amount of CRP suggest an acute infection on inflammation
c-reactive protein
53
* Most plasma proteins are synthesized in the (?) and secreted by the (?) into the circulation.
* The immunoglobulin are exceptions because they are synthesized in (?)
* The nitrogen content of serum proteins is, on average (?)%
* (?): site of protein synthesis within the cell
* liver, hepatocytes
* plasma cells
* 16
* ribosomes
54
# globulin
* Function: scavenge heme released or lost by the turnover of heme proteins such as hemoglobin → protect bocy from oxidative damage that free heme can cause
* Low levels are diagnostic of hemolytic anemia
hemopexin
55
# complement
most abundant complement protein in human serum
C3
56
# globulin
* Function: bind free hemoglobin to prevent loss of hemoglobin and its constituent, iron, into the urine
* synthesized in the liver as a tetramer consisting of two α and two β chains.
* Used primanly to help and evaluate hemolytic anemia
haptoglobin
57
58
# levels/structure
It is the actual three-dimentional configuration; the folding pattern of proteins
tertiary
