Biomaterial Database

Urinary proteins in a patient with Tangier disease. 1985

P H Pritchard, and M Bergseth, and R McLeod, and M R Hayden, and J Frohlich

We have investigated the possibility that the mild proteinuria in a patient with Tangier disease is directly related to the plasma HDL-deficiency through excretion of apolipoprotein A-I (Mr 28,300). An increased urinary excretion of a 29,000 polypeptide was observed in this patient. However, western blot analysis of the urine showed that this protein was not apolipoprotein A-1 or its precursor form. Subsequent investigations identified the urinary protein as immunoglobulin light chains. The elevated excretion may be a consequence of the patient's plasma polyclonal gammopathy which had resulted from a chronic infection.

UI	MeSH Term	Description	Entries
D007009	Hypolipoproteinemias	Conditions with abnormally low levels of LIPOPROTEINS in the blood. This may involve any of the lipoprotein subclasses, including ALPHA-LIPOPROTEINS (high-density lipoproteins); BETA-LIPOPROTEINS (low-density lipoproteins); and PREBETA-LIPOPROTEINS (very-low-density lipoproteins).	Hypolipoproteinemia,Hypoprebetalipoproteinemia
D007147	Immunoglobulin Light Chains	Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. There are two major types of light chains, kappa and lambda. Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule.	Ig Light Chains,Immunoglobulins, Light-Chain,Immunoglobulin Light Chain,Immunoglobulin Light-Chain,Light-Chain Immunoglobulins,Chains, Ig Light,Chains, Immunoglobulin Light,Immunoglobulins, Light Chain,Light Chain Immunoglobulins,Light Chain, Immunoglobulin,Light Chains, Ig,Light Chains, Immunoglobulin,Light-Chain, Immunoglobulin
D008075	Lipoproteins, HDL	A class of lipoproteins of small size (4-13 nm) and dense (greater than 1.063 g/ml) particles. HDL lipoproteins, synthesized in the liver without a lipid core, accumulate cholesterol esters from peripheral tissues and transport them to the liver for re-utilization or elimination from the body (the reverse cholesterol transport). Their major protein component is APOLIPOPROTEIN A-I. HDL also shuttle APOLIPOPROTEINS C and APOLIPOPROTEINS E to and from triglyceride-rich lipoproteins during their catabolism. HDL plasma level has been inversely correlated with the risk of cardiovascular diseases.	High Density Lipoprotein,High-Density Lipoprotein,High-Density Lipoproteins,alpha-Lipoprotein,alpha-Lipoproteins,Heavy Lipoproteins,alpha-1 Lipoprotein,Density Lipoprotein, High,HDL Lipoproteins,High Density Lipoproteins,Lipoprotein, High Density,Lipoprotein, High-Density,Lipoproteins, Heavy,Lipoproteins, High-Density,alpha Lipoprotein,alpha Lipoproteins
D008297	Male		Males
D008970	Molecular Weight	The sum of the weight of all the atoms in a molecule.	Molecular Weights,Weight, Molecular,Weights, Molecular
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D001054	Apolipoproteins A	Structural proteins of the alpha-lipoproteins (HIGH DENSITY LIPOPROTEINS), including APOLIPOPROTEIN A-I and APOLIPOPROTEIN A-II. They can modulate the activity of LECITHIN CHOLESTEROL ACYLTRANSFERASE. These apolipoproteins are low in atherosclerotic patients. They are either absent or present in extremely low plasma concentration in TANGIER DISEASE.	Apo-A,ApoA
D013631	Tangier Disease	An autosomal recessively inherited disorder caused by mutation of ATP-BINDING CASSETTE TRANSPORTERS involved in cellular cholesterol removal (reverse-cholesterol transport). It is characterized by near absence of ALPHA-LIPOPROTEINS (high-density lipoproteins) in blood. The massive tissue deposition of cholesterol esters results in HEPATOMEGALY; SPLENOMEGALY; RETINITIS PIGMENTOSA; large orange tonsils; and often sensory POLYNEUROPATHY. The disorder was first found among inhabitants of Tangier Island in the Chesapeake Bay, MD.	A-alphalipoprotein Neuropathy,Analphalipoproteinemia,Tangier Disease Neuropathy,Alpha High Density Lipoprotein Deficiency Disease,Cholesterol Thesaurismosis,HDLDT1,High Density Lipoprotein Deficiency, Tangier Type,High Density Lipoprotein Deficiency, Type 1,High-Density Lipoprotein Deficiency, Tangier Type,High-Density Lipoprotein Deficiency, Type I,Neuropathy of Tangier Disease,Tangier Hereditary Neuropathy,A-alphalipoprotein Neuropathies,Analphalipoproteinemias,Cholesterol Thesaurismoses,High Density Lipoprotein Deficiency, Type I,Neuropathies, A-alphalipoprotein,Neuropathy, A-alphalipoprotein,Thesaurismoses, Cholesterol,Thesaurismosis, Cholesterol
D016632	Apolipoprotein A-I	The most abundant protein component of HIGH DENSITY LIPOPROTEINS or HDL. This protein serves as an acceptor for CHOLESTEROL released from cells thus promoting efflux of cholesterol to HDL then to the LIVER for excretion from the body (reverse cholesterol transport). It also acts as a cofactor for LECITHIN CHOLESTEROL ACYLTRANSFERASE that forms CHOLESTEROL ESTERS on the HDL particles. Mutations of this gene APOA1 cause HDL deficiency, such as in FAMILIAL ALPHA LIPOPROTEIN DEFICIENCY DISEASE and in some patients with TANGIER DISEASE.	Apo A-I,Apo A-1,Apo A-I Isoproteins,Apo A1,Apo AI,ApoA-1,ApoA-I,Apolipoprotein A-1,Apolipoprotein A-I Isoprotein-2,Apolipoprotein A-I Isoprotein-4,Apolipoprotein A-I Isoproteins,Apolipoprotein A1,Apolipoprotein AI,Apolipoprotein AI Propeptide,Pro-Apo A-I,Pro-Apolipoprotein A-I,Proapolipoprotein AI,Apo A I Isoproteins,Apolipoprotein A 1,Apolipoprotein A I,Apolipoprotein A I Isoprotein 2,Apolipoprotein A I Isoprotein 4,Apolipoprotein A I Isoproteins,Pro Apo A I,Pro Apolipoprotein A I

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