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Proteins

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1
Amino acids that must be supplied by dietary intake
Essential amino acids
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Enumeration of essential amino acids (MPTTYVHILL)
Methionine, Phenylalanine, Threonine, Tryptophan, Valine, Histidine, Isoleucine, Leucine, Lysine
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Product formed by the removal of an amino group from an amino acid
Ketoacids
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The two general classifications of proteins
Simple or conjugated
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Simple proteins are defined as peptide chains that hydrolyze to
amino acids
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Conjugated proteins are composed of a protein (apoprotein) and a
nonprotein substance (prosthetic groups)
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The prosthetic group that forms lipoproteins
lipid
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The prosthetic group that forms glycoproteins
carbohydrate
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The prosthetic group that forms metalloproteins
metals
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Four amino acids componenrs are
Amino group, carboxyl group, hydrogen, side chain (R-group)
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Amino acid groups
essential amino acids, nonessential amino acids, conditional amino acids
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Component that gives alpha-amino acids their individual special properties
Side chain (R-group)
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Macro-molecules composed of covalently linked polymers of amino acids linked by peptide bonds in a head-to-tail fashion
Proteins
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Two common sites for protein synthesis
Liver and plasma cells
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Characteristics of Type I DM patients
Thin, ketosis-prone, genetic predisposition (Ag HLA-DR3, DR4, DQ)
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Primary treatment for Type I DM
insulin therapy
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Characteristics of Type II DM ("NIDDM")
Obese patients, ketosis usually absent, manifests after 40 years of age
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Cause of Type II DM
absent or defective insulin receptors
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Type of Diabetes Mellitus linked to Alzheimer's disease
Type III DM
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Type of Diabetes Mellitus caused by malnutrition
Type V DM
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C-peptide status in Type I vs Type II DM
Type I = C peptide (-); Type II = C peptide (+)
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Source and structure of C-peptide
31-aa peptide from the pancreas connecting alpha and beta chains of proinsulin
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Glycogen storage disease Ia name and deficiency
Von Gierke; Glucose-6-Phosphatase deficiency
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Glycogen storage disease II name and deficiency
Pompe; Alpha-1,4-glucosidase deficiency
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Glycogen storage disease III name and deficiency
Cori-forbes; Debrancher enzyme deficiency
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Glycogen storage disease IV name and deficiency
Andersen; Brancher enzyme deficiency
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Glycogen storage disease V name and deficiency
Mc Ardle; Muscle phosphorylase deficiency
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Glycogen storage disease VI name and deficiency
Hers; Liver phosphorylase deficiency
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Glycogen storage disease VII name and deficiency
Tarui; Phosphofructokinase deficiency
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Glycogen storage disease XI name and deficiency
Fanconi Bickel; Glucose transporter 2 deficiency
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Checkpoint: Enzyme deficiency in GSD Ib
Glucose-6-phosphatase translocase
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Checkpoint: Enzyme deficiency in GSD IIIb
Glycogen debranching enzyme in liver only
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Checkpoint: Enzyme deficiency in GSD IXa
phosphorylase kinase def in liver
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Checkpoint: Enzyme deficiency in GSD Vb
phosphorylase kinase def in liver and muscle
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Checkpoint: Enzyme deficiency in GSD Type 0
Glycogen synthase def
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Two primary products of protein catabolism
ammonia and ketoacids
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Chemical needed in the formation of urea
ammonia
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Conditions characterized by hyperproteinemia
dehydration, chronic inflammation, and multiple myeloma
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Associated proteins or markers for hyperproteinemia
CHON, APRs (acute phase reactants), and light chains
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Conditions characterized by hypoproteinemia
nephrotic syndrome, malabsorption, overhydration, hepatic insufficiency, malnutrition, and agammaglobulinemia
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Total protein loss per day in nephrotic syndrome
> 3.5 g of protein
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The identification of Bence Jones protein is best accomplished by
immunofixation or urine protein electrophoresis
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Screening method for Bence Jones protein involving heat
Urine Heat Test at 40-60°C
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The Urine Heat Test is positive for Bence Jones protein because it
turbidly precipitates
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Proteolytic fragments of collagen I formed during bone resorption (turnover)
Crosslinked telopeptides
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Biochemical marker of bone resorption detected in serum and urine
CTX
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The bone turnover rate (CTX) increases during
menopause or osteoporosis
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Non-collagenous protein in bone synthesized by osteoblasts
Osteocalcin
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Markers that reflect osteoblast activity or bone formation
Osteocalcin levels
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Protein that demonstrates a wide variety of cellular interactions including cell adhesion and wound healing
Fibronectin
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Protein that has an inverse correlation with body mass index (BMI)
Adiponectin
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Conditions correlated with lower levels of Adiponectin
heart disease, type 2 diabetes, metabolic syndrome, and obesity
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Verified accurate marker for CSF leakage
B-trace protein (BTP)
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Potential marker for detecting impaired renal function, though not more sensitive than cystatin C
B-trace protein (BTP)
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New sensitive endogenous serum marker for the glomerular filtration rate (GFR)
Cystatin C
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Gold standard for measuring GFR
Inulin
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Major advantage of Cystatin C over Inulin for GFR measurement
it is endogenous
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Cardiac markers found in cardiac and skeletal muscle with different gene encoding
Troponin I and Troponin T
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Most specific marker for Acute Coronary Syndrome (ACS/MI)
Cardiac troponins (specifically Trop I and Trop T)
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Troponin subunit that acts as an inhibitory protein and is highly specific
Trop I
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Troponin subunit that is a tropomyosin binding protein
Trop T
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Troponin subunit that is a calcium binding protein and is less specific
Trop C
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Primary oxygen carrying protein in the striated muscle and most sensitive marker for AMI
Myoglobin
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Stain used to identify Amyloid
Congo red
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Protein that may infiltrate many organs causing localized or widespread organ failure
Amyloid
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Protein absent in serum electrophoresis because it has been converted into a fibrin clot
Fibrinogen
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Major proteins in plasma that contribute to the electrophoretic pattern
albumin, α1-antitrypsin, α2-macroglobulin, haptoglobin, β-lipoprotein, transferrin, complement, C3, fibrinogen, and immunoglobulins
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Transport protein that carries and releases Iron (Fe) to the bone marrow
Transferrin
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Trace element also transported by Transferrin
Chromium
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Clinical condition where Transferrin levels are usually increased
Iron Deficiency Anemia (IDA)
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Transport protein for T4 and T3
TBG (Thyroxine-binding globulin), Albumin, and TBP (prealbumin)
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Transport protein for Cortisol
Transcortin
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Transport protein for Lipids (cholesterol and TAG)
Lipoproteins
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Transport protein for Copper
Ceruloplasmin
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Transport protein for Hemoglobin
Haptoglobin
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Transport protein for H+ and CO2
Hemoglobin
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Protein that binds excess free heme
Hemopexin
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Major plasma protein responsible for maintaining oncotic pressure
Albumin
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Substances bound and transported by Albumin
bilirubin, steroids, and fatty acids
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Bilirubin transport in plasma is primarily handled by
Albumin
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Proteins involved in the transport of bilirubin in the liver
Y and Z proteins (ligandin and FABP)
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Indicator of nutrition that binds thyroid hormones and retinol-binding protein
Prealbumin
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Marker used to identify short term malnutrition (after 2 days)
Prealbumin
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Marker used to identify long term malnutrition (20 days)
Albumin
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Principal fetal protein where elevated levels indicate risk for spina bifida and NTD (neural tube defects)
AFP (Alpha-fetoprotein)
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Handwritten clinical significance of AFP besides fetal defects
Hepatocellular tumor or liver cancer
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Two types of Neural Tube Defects (NTD) associated with AFP
anencephaly (common) and spina bifida
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Acute-phase reactant that serves as a protease inhibitor by inhibiting neutrophil elastase
Alpha-1-antitrypsin
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Protein that transports Vitamin D and binds actin
Gc globulin
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Acute Phase reactant with oxidase activity that contains copper
Ceruloplasmin
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Lipoprotein that transports lipids, primarily VLDL triglyceride
Pre-beta lipoprotein
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Lipoprotein that transports lipids, primarily LDL cholesterol
Beta lipoprotein
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Acute-phase reactant that motivates phagocytosis in inflammatory disease
CRP (C-reactive protein)
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The most sensitive marker for inflammation and the 1st to rise during infection
CRP
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Timeframe when CRP typically shows after infection or inflammation
6-48 hours
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The extra band seen between beta and gamma in an electrophoretic pattern represents
Fibrinogen
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Electrophoretic pattern showing increased alpha-1 and alpha-2 fractions
Acute inflammation
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Electrophoretic pattern showing increased alpha-1, alpha-2, and gamma with decreased albumin
Chronic infection
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Electrophoretic pattern with a polyclonal increase in all gamma fractions and beta-gamma bridging
Hepatic cirrhosis
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Pattern with a sharp increase in one immunoglobulin (M spike) and a decrease in other fractions
Monoclonal gammopathy
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Specific clinical example of a Monoclonal gammopathy
Multiple Myeloma (MM)
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Pattern with a diffuse increase in gamma without bridging
Polyclonal gammopathy
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Clinical examples of polyclonal gammopathy
SLE and Rheumatoid Arthritis (RA)
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Electrophoretic pattern characterized by decreased gamma fractions
hypogammaglobulinemia/agammaglobulinemia
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Specific example of decreased gamma involving Bruton's
Bruton's agammaglobulinemia
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Pattern showing decreased albumin and increased alpha-2
protein losing nephropathy/Nephrotic syndrome
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Specific lab findings in nephrotic syndrome (PLNS)
Albumin (decreased) and Alpha-2 macroglobulin (increased)
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Electrophoretic pattern showing decreased alpha-1 fraction
AAT deficiency or juvenile cirrhosis
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Electrophoretic pattern with a beta or unusual band between alpha-2 and beta
Hemolysis
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The part of an amino acid structure that determines its specificity
R-group (side chain)
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The four components of an amino acid chemical structure
Amino group (NH_2), Carboxyl group (COOH), Hydrogen atom (H), and R-group
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The process of removing the amino group from an amino acid
Deamination
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The two products of deamination
Ammonia and Ketoacid
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Definition of a Conjugated Protein
Apoprotein + Prosthetic group
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Result of Apoprotein + Carbohydrate (CHO)
Glycoprotein
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Result of Apoprotein + Lipids
Lipoprotein
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Result of Apoprotein + Metals
Metalloprotein
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Classification of Erythropoietin
Glycoprotein
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Classification of Hemoglobin (Hgb)
Metalloprotein
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Classification of Casein
Phosphoprotein
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Classification of Albumin based on structure
Simple protein
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Marker used specifically for bone resorption
Crosslinked telopeptides (CTX)
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Clinical condition indicated by increased CTX levels
Osteoporosis (bone destruction)
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Hormone whose decline is linked to increased bone turnover in women
Estrogen
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Marker that reflects both bone formation and bone resorption
Osteocalcin
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The first marker (protein) to rise in Acute Myocardial Infarction (AMI)
Myoglobin
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The first enzyme to rise in AMI
CK-MB
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The most specific enzyme for the heart
CK-MB
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The most specific substance (marker) for heart damage
Troponin I (Trop I)
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Two enzymes historically used as cardiac markers
AST and LDH
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The last enzyme to rise and normalize following an AMI
LDH
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The tissue source for CK-MM (CK-3)
Skeletal and heart muscle
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The tissue source for CK-MB (CK-2)
Heart
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The tissue source for CK-BB (CK-1)
Brain
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Specific name for CK-M
Mitochondrial CK
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