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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
1987-4-6
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pubmed:abstractText |
Serum-denatured TBG (dnTBG) measured in 32 families deficient in native TBG (nTBG) was undetectable in all subjects with complete nTBG deficiency and was high in 2 of 16 families with partial nTBG deficiency. nTBG (in mean micrograms per decaliter +/- SD) in members of the Quebec and Montreal families, respectively were: 258 +/- 54 and 230 in affected men, 747 +/- 190 and 927 +/- 90 in affected women, and 1568 +/- 151 and 1300 +/- 195 in unaffected relatives. Corresponding mean dnTBG levels were: 14.3 +/- 2.9 and 21.3 in affected men, 8.6 +/- 1.0 and 11.6 +/- 3.1 in affected women, and less than 2.1 and less than 2.6 in unaffected relatives. All were euthyroid with normal free thyroxine and thyrotropin levels. In comparison to common type TBG, TBG-Quebec was more heat labile by 10 degrees C and TBG-Montreal by 12 degrees C. The degree of dnTBG elevation and nTBG lability at 37 degrees C were correlated (r = 0.99). Isoelectric focusing showed cathodal shift of all TBG bands: TBG-Quebec by 0.06 isoelectric points (pI) and TBG-Montreal by 0.02 pI. These two TBG variants represent different mutations most likely affecting the polypeptide chain of the molecule. Their inheritance is X-chromosome linked. The instability of these TBGs at 37 degrees C may lead to more rapid degradation in vivo resulting in low nTBG and high dnTBG concentrations in serum.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-13798328,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-216060,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-3081330,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-3081807,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-3086361,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-3917459,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-3928734,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-402376,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-4111366,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-4171474,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-4621446,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-5006665,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-56342,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6162169,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6415085,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6429183,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6782114,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6789676,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6794564,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-6794959,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3102557-773572
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
0021-9738
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
79
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
833-40
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:3102557-Female,
pubmed-meshheading:3102557-Genetic Variation,
pubmed-meshheading:3102557-Hot Temperature,
pubmed-meshheading:3102557-Humans,
pubmed-meshheading:3102557-Hydrogen-Ion Concentration,
pubmed-meshheading:3102557-Isoelectric Focusing,
pubmed-meshheading:3102557-Male,
pubmed-meshheading:3102557-Pedigree,
pubmed-meshheading:3102557-Protein Denaturation,
pubmed-meshheading:3102557-Thyroxine,
pubmed-meshheading:3102557-Thyroxine-Binding Proteins,
pubmed-meshheading:3102557-Triiodothyronine,
pubmed-meshheading:3102557-Triiodothyronine, Reverse
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pubmed:year |
1987
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pubmed:articleTitle |
Two new inherited defects of the thyroxine-binding globulin (TBG) molecule presenting as partial TBG deficiency.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.
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