Total Protein Lecture Flashcards
Protein characteristics
carbon, nitrogen, hydrogen & oxygen
peptide bonds between the NH2 & the carboxyl group of 2 amino acids
zwitterion/ ampholytic property
~16% nitrogen
Zwitterion/ampholytic property
can take on a negative or positive charge depending on the pH being above or below an isoelectric point of the amino acid (pI)
Protein structure
primary - chain
secondary- pleated sheets & alpha helix
tertiary
quaternary- multi protein complex
Cellular protein synthesis
stimulating hormone acts on an active metabolite which will act on the nucleus & stimulate production of mRNA for certain protein to either be packaged and sent to other cells or remain in the cell
PKU aminoacidopathy
lack of phenylalanine hydroxylase -> build up of phenylalanine in the blood and can cause mental retardation in children & damages the brain
Tyrosinemia aminoacidopathy -3 types
type 1: fumarylacetoacetate hydrolase type 2: tyrosine aminotransferase (most common) - build of tyrosine that can be damaging to a child's development type 3 (rare): 4-hydroxyphenylpyruvate dioxygenase
Alkaptonuria aaminoacidopathy
lack homogentisate oxidase & causes an accumulation of HGA & when urine is exposed to the atmosphere it will turn black (very dark)
Maple Syrup Disease (MSUD) aminoacidopathy
decreased or absent branched chain a-ketoacid decarboxylase
this enzyme is important in metabolizing branched chain amino acids & causes them to build up & be excreted through the urine & smells like maple syrup
Isovaleric Acidemia aminoacidopathy
isovaleryl-Co-A dehydrogenase
Homocystinuria aminoacidopathy
cystathionine-beta-synthetase w/o this enzyme you can get a build up of the precursor
Citrullinemia aminoacidopathy
type 1: argininosuccinic acid synthetase
Argininosuccinic Aciduria aminoacidopathy
lack of arginosuccinic acid lyase
Cystinuria aminoacidopathy
defect in amino acid transport system so that kidneys do not filter cystine & precipitates in the urine
Protein Functions
energy osmotic force - albumin acid-base balance transport antibodies hormones enzymes hemostasis
total protein
range: 6.4-8.3 g/dL
2 groups:
albumin & globular proteins
other tissue proteins should not normally be found in the blood
Albumin general
3.4-5.0 g/dL
most abundant serum protein
transport protein
osmotic balance
globulin general
2.5-4.2 g/dL
many specific proteins in this group, differentiated from electrophoresis
Pre-albumin (Transthyretin)
transport protein for T4 & T3
binds retinol-binding protein to transport vitA (special interest to nutritionists)
Albumin (specific)
small protein w/ highest concentration in the body
80% of colloid osmotic pressure
buffers pH
negative acute phase reactant (decreases during stress/inflammation)
alpha 1 globulins
alpha-1-antitrypsin (AAT)
alpha-1-fetoprotein (AFP)
alpha-1-acid glycoprotein (orosomucoid)
alpha-1-antichymotrypsin
alpha-1-antitrypsin (AAT)
inhibits neutrophil elastase (Protease)
increased in acute phase reaction, pregnancy, & contraception meds
alpha-1-fetoprotein (AFP)
synthesized in the embryonic development
binds to estradiol
low AFP in mom can indicate Down’s syndrome