Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Concentrations of free amino acids were measured concurrently in plasma, erythrocytes, granulocytes, and lymphocytes in umbilical cord blood, neonates, children, and adults. In each age group, the patterns of free amino acids were fairly similar in plasma and erythrocytes except for aspartic acid which was more abundant in erythrocytes. Of the amino acids in granulocytes, 71-77% was taurine; in lymphocytes taurine, aspartic acid, and glutamic acid comprised 35-44%, 18-24%, and 20-28%, respectively, of the total in all age groups. Leukocytes may contribute to the interorgan transport of amino acids to about 10% of the erythrocytes' contribution. Postnatally, the levels of glutamic acid and tyrosine in plasma; threonine plus glutamine, serine plus asparagine, and tyrosine in erythrocytes; histidine in granulocytes; and glutamic acid in lymphocytes were significantly increased (p less than 0.001); while the levels of phenylalanine and lysine in plasma; taurine in erythrocytes; valine and phenylalanine in granulocytes; and threonine plus glutamine, tyrosine, and phenylalanine in lymphocytes were significantly decreased (p less than 0.001). After the neonatal period concentrations of taurine and aspartic acid in erythrocytes, taurine and valine in granulocytes, and tyrosine and phenylalanine in lymphocytes increased gradually with age; while concentrations aspartic acid in plasma, histidine in granulocytes, and glycine in lymphocytes decreased gradually with age. The levels of glycine and valine in plasma, alanine and valine in erythrocytes, serine plus asparagine, glycine, alanine, and tyrosine in granulocytes, and aspartic acid, serine plus asparagine, and alanine in lymphocytes remained constant in all age groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Free amino acid concentrations in plasma, erythrocytes, granulocytes, and lymphocytes in umbilical cord blood, children, and adults. 673 89

Endoplasmic reticulum (ER)-resident proteins are continually retrieved from the Golgi and returned to the ER by Lys-Asp-Glu-Leu (KDEL) receptors, which bind to an eponymous tetrapeptide motif at their substrate's C terminus. Mice and humans possess three paralogous KDEL receptors, but little is known about their functional redundancy, or if their mutation can be physiologically tolerated. Here, we present a recessive mouse missense allele of the prototypical mammalian KDEL receptor, KDEL ER protein retention receptor 1 (KDELR1). Kdelr1 homozygous mutants were mildly lymphopenic, as were mice with a CRISPR/Cas9-engineered frameshift allele. Lymphopenia was cell intrinsic and, in the case of T cells, was associated with reduced expression of the T-cell receptor (TCR) and increased expression of CD44, and could be partially corrected by an MHC class I-restricted TCR transgene. Antiviral immunity was also compromised, with Kdelr1 mutant mice unable to clear an otherwise self-limiting viral infection. These data reveal a nonredundant cellular function for KDELR1, upon which lymphocytes distinctly depend.
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PMID:Mutation of the ER retention receptor KDELR1 leads to cell-intrinsic lymphopenia and a failure to control chronic viral infection. 2643 36