| Term | Definition |
|
Amino aciduria |
This condition has primary and secondary causes that include inherited genetic defect and renal tubular dysfunction. |
|
Inherited genetic defect (autosomal recessive) |
>70% of amino acidurias are caused by this and are referred to as primary causes. |
|
Metabolite accumulation |
A primary cause for amino aciduria can lead to... |
|
Liver damage (causing over flow) and renal tubular dysfunction |
The two secondary causes for amino aciduria. |
|
Phenylketonuria |
This disease is marked by a phenylalanine hydroxylase deficiency leading to the accumulation of phenylalanine and toxic products. |
|
Severe mental retardation |
This is a consequence of not treating a baby with phenylketonuria. |
|
Diet without phenylalanine (including treatment with amino acid-tyrosine to make up for inability to produce it from phenylalanine) |
Treatment for phenylketonuria. |
|
Globular proteins |
These proteins are small spheres with little to no water inside. They have hydrophobic amino acids in the inside and hydrophilic R groups on the outside. |
|
pH, temperature |
Tight range of ___________ and ____________ stability are needed or else the globular proteins will denature. |
|
Amino acid sequence |
The primary structure of a protein is made up of... |
|
Alpha helix or Beta pleated sheets |
The secondary structure of proteins consists of... |
|
Polypeptides that fold in on themselves |
The tertiary structure of proteins consist of... |
|
Subunit aggregation (with or without prosthetic groups) |
The quaternary structure of proteins consists of... |
|
Ammonium sulfate |
This compound aids in the "salting out" of soluble serum proteins. |
|
Liver |
Most serum proteins are produced here. |
|
Sialic acid |
The part of the RBC membrane that gives it a negative charge. |
|
CRP |
This protein is the first indication of inflammation in the body. |
|
Retinol-binding protein (RBP) and Transthyretin (TTR) |
Prealbumin consists of... |
|
Prealbumin |
This can often be a more sensitive indicator of protein malnutrition or liver dysfunction. |
|
Prealbumin |
This protein's level goes down when there is inflammation or malignancy. |
|
Retinol-Binding Protein |
This protein's levels go up in liver dysfunction, protein malnutrition, and chronic renal disease. |
|
Zinc deficiency |
This is characterized by a low RBP and low total vitamin A. |
|
3.5-5.0 g/dL |
Reference range for Albumin. |
|
6-8 g/dL |
Reference range for total protein. |
|
Albumin |
This protein is the first protein lost through the glomerulus. |
|
Albumin |
This protein goes up in dehydration. |
|
Albumin |
This protein goes down in inflammation, nephrotic syndrome, GI loss, and malnutrition. |
|
Amniotic fluid and mother's serum |
Analyzed to find alpha1 fetoprotein. |
|
Spina bifida |
Alpha1-Fetoprotein is used to study neural tube defects that are indicative of... |
|
RIA |
This test is used to analyze alpha1 fetoprotein. |
|
Increase |
Burns and nephrotic syndrome cause a/n ________________ in haptoglobin. |
|
Decrease |
Intravascular hemolysis, transfusion reactions and malaria cause a/n ______________ in haptoglobin. |
|
1 week |
How long it takes for haptoglobin levels to return to normal after hemolysis depletion. |
|
3g |
During intravascular hemolysis haptoglobin can take on up to _________ of hemoglobin. |