increase with infections and inflammation

    decrease alpha 1-antitrypsin deficiency   

Bind free hemoglobin

Transport Copper

Neutralize trypsin, plasmin, hormones

Transport lipoprotein/ Fe, bind Heme, immunity, clotting

Principle: monoclonal antibodies developed toward a specific protein in a band is used to detect the amount of protein in blood, CSF, or urine.

Nephelometry

Turbidimetry

ELISA

proteins that precipitate at cold temperatures when blood circulates to the extremities (Raynaud’s Phenomenon) and are produced by most patients with

Waldenstrom’s macroglobulinemia (IgM)

SLE

RA

Polycythemia

Specimen is placed on separate tracks in an agarose gel or on cellulose acetate strips. The proteins are separated by electrophoresis. One track is treated with a fixative that fixes all proteins, creating a reference pattern. The other tracks are treated with monospecific antisera to IgG, IgM, IgA and kappa and lambda.

•Newest method of separation based on movement of a molecule in an electric field

Efficient heat transfer that allows high voltage, rapid separation and high resolution

–Detection window in capillary measures light absorbed at 214 nm by peptide bonds giving a direct concentration of protein in sample

•High resolution produces distinct transferrin and complement bands in the beta region

characteristic of renal disease and concentrations may be increased with diabetes, hypertension, nephrotic syndrome, and drug nephrotoxicity

: “Light chain" portion of immunoglobulin protein excreted in the urine of myeloma patients

________produce a fine suspension of CSF protein particles

Decreased CSF Protein is usually seen when CSF is leaking from the CNS

•Main purpose: detection of oligoclonal IgG bands

•Oligoclonal bands are immunoglobulins(proteins)

–The presence of these only in the CSF are used in the diagnosis of Multiple Sclerosis.

•Stains:

–Coomassie Brilliant Blue

–Silver Stain

Aminoacidopathies

Rare inherited disorders of amino acid metabolism

•Phenyalanine levels accumulate in serum

•Phenylpyruvic acid excreted in urine (a “phenylketone”)

Aminoacidopathies

Newborn screening card – obtained at discharge of baby; heelstick done.

–Can cause mental retardation if not detected

–Dietary restrictions put in place to eliminate all phenylalanine from the diet.

–As the child grows, an alternate phenylalanine pathway develops, and the dietary restrictions are eased.

Aminoacidopathies

-Can be inherited or a metabolic defect

-Produce tyrosine and leucine crystals in the urine.

-Hereditary defects are usually fatal.

Aminoacidopathies

-Genetic defect which results in failure to produce homogentisic acid oxidase. This -causes homogentisic acid to accumulate in the blood and urine.

-Produces brown pigment deposits in the body tissue which can lead to arthritis, liver and cardiac problems.

Aminoacidopathies

Fatal- unique smell association

-Caused by low levels of branched chain keto acid decarboxylase.  This inhibits metabolism of leucine, isoleucine, and valine.

-Untreated it causes severe mental retardation, convulsions, acidosis, and hypoglycemia.