8) Proteins Flashcards
basic protein formula
RCH(NH2)COOH
isoelectric point (pI)
the pH at which the amino acid or protein has no net charge, and the positive charges equal the negative charges
At a pH ——– than the pI, the protein carries a ——– charge
greater – negative
less – positive
peptide bond
Chemical bond between a carboxyl group of one molecule and an amino group of the other molecule, releasing water
Plasma ——— provided by the proteins tends to retain water in the vascular space
colloid osmotic pressure (COP)
3 main functions of plasma proteins
- Maintenance of water distribution between cells and tissue.
- Coagulation proteins are important in maintenance of hemostasis.
- Transport vehicles to move various ligands to where they are needed or stored.
Rare inherited disease involving the homogentisic acid oxidase
Leads to a buildup of homogentisic acid in the tissues of the body
alkaptonuria
darkening of the tissues of the body because of the excess homogentisic acid
Ochronosis
Caused by the absence or very low levels of the branched-chain enzyme α-keto acid decarboxylase complex
MSUD
Results in the abnormal metabolism of three essential amino acids:
leucine, isoleucine, and valine
MSUD
Results in the inability to metabolize the essential amino acid phenylalanine
phenylketonuria
grouping of plasma proteins
- Albumin
- Globulin (α1 globulins, α2 globulins, ß1 globulins, λ globulins)
TP =
albumin + globulin
Main clinical significance of ———- is role as a sensitive marker of poor nutritional status such as protein-energy malnutrition (PEM).
prealbumin/transthyretin (TTR)
protein measured in CSF, not electrophoresis
prealbumin/TTR
albumin is approximiately —-% of serum protein
60
list the 16 plasma proteins
𝛼1 - 3
𝛼2 - 3
β - 5
𝛾 - 2
prealbumin
albumin
—
𝛼1-antitrypsin
𝛼1-acid glycoprotein
alpha-fetoprotein
—
haptoglobin
ceruloplasmin
𝛼2-macroglobulin
—
transferrin
hemopexin
β-lipoproteins
β2-microglobulin
C3
—
fibrinogen
—
C-reactive protein
Ig
albumin functions
Maintain plasma colloid osmotic pressure.
Bind and transport a wide variety of ligands.
Serve as an endogenous source of amino acids.
causes of hypoalbuminemia
- increased catabolism (most common)
- impaired synthesis
- increased protein loss
- analbuminemia
causes of hyperalbuminemia
- Dehydration/decreased plasma volume
- high protein diet
- albumin infusion
A/G ratio
albumin/globulin
globulin = TP - albumin
RR for A/G ratio
0.8-2.2
Major α1–globulin, making up approximately 90% of α1–proteins.
α1–Antitrypsin (AAT)
one of the most common genetically lethal diseases in Caucasians (1:4000)
AAT deficiency
sx of AAT deficiency
emphysema with onset at 45 years of age or earlier
emphysema occurring in the absence of smoking
is the major glycoprotein increased during inflammation, an Acute Phase Reactant (APR).
α1–Acid Glycoprotein/orosomucoid
(AAG)
Principal fetoprotein (fetal albumin-like protein) in maternal serum
alpha-fetoprotein
Used to screen for the antenatal diagnosis of neural tube defects including spina bifida and anencephaly
AFP
—— is decreased in Down’s syndrome and Trisomy 18
AFP
increased levels found in 80% of patients with hepatocellular cancer, 50% of germ cell tumors (gonadal), and all children with hepatoblastoma
AFP
An acute phase reactant that binds free hemoglobin in plasma
haptoglobin (Hp)
Depletion is the most sensitive indicator of intravascular hemolysis, in transfusion reactions, and certain hemolytic anemias.
Hp
Principal copper (Cu)-containing protein in plasma containing 95% of the total serum copper
Ceruloplasmin (Cp)
Decreased in Wilson’s disease, Menkes disease, malnutrition, malabsorption, severe liver disease, nephritic syndrome
Cp
Rare autosomal recessive trait where Cp levels are reduced and the dialyzable Cu concentration is increased.
Wilson disease
copper chealators used to tx Wilson’s
penicillamine
trientine
effects of Wilson’s disease
- Degenerative cirrhosis
- Chronic active hepatitis
- Renal tubular acidosis
- Neurological damage (clumsiness, tremors)
- Kayser-Fleischer rings, pigmented rings at the outer margins of the cornea and the sclera
In nephrotic syndrome, —— is characteristically increased above normal because it is retained while smaller proteins are excreted in the urine.
AMG
Major component of β-globulins
negative acute phase reactant
TRF
Principal plasma protein for transport of iron as Fe+3 — the ferric ion — to storage sites, where it is bound to apoferritin and stored as ferritin
TRF
Important in the differential diagnosis of anemias and monitoring the treatment of iron deficiency anemia
TRF
A commonly used indicator of iron overload—screen for hemochromatosis
TRF
When red blood cells are destroyed, it transports heme to the liver, where it is catabolized by the reticuloendothelial system.
hemopexin
Increased levels are found in pregnancy and in diabetes mellitus.
hemopexin