Harpers

672 / INDEXLocus control regions, 387Long interspersed repeat sequences(LINEs), 321–322Looped domains, chromatin, 316, 318,319fLoops (protein conformation), 32–33Loose connective tissue, keratan sulfate I in,545Low-density lipoprotein receptor-relatedprotein, 206in chylomicron remnant uptake,208–209, 209fLow-density lipoproteins, 205, 206tapolipoproteins of, 206, 206tmetabolism of, 209, 210fratio of to high-density lipoproteins,atherosclerosis and, 227receptors for, 209in chylomicron remnant uptake,208–209, 209fin cotranslational insertion, 505–506,506fregulation of, 223Low-energy phosphates, 83β-LPH. See β-LipotropinLRP. See Low-density lipoproteinreceptor-related proteinL-tryptophan dioxygenase (tryptophanpyrrolase), 89LTs. See LeukotrienesLung surfactant, 115, 197deficiency of, 115, 202Luteinizing hormone (LH), 437, 438, 439fLXs. See LipoxinsLXXLL motifs, nuclear receptorcoregulators, 473Lyases, 50in steroid synthesis, 440–442, 441f,443fLymphocyte homing, selectins in, 528–530,529f, 529t, 530fLymphocytes. See also B lymphocytes;T lymphocytesrecombinant DNA technology in studyof, 624Lysine, 16tcatabolism of, 256f, 258pI of, 17requirements for, 480Lysine hydroxylase, vitamin C as coenzymefor, 496Lysis, cell, complement in, 596Lysogenic pathway, 379, 379fLysolecithin (lysophosphatidylcholine),116, 116fmetabolism of, 200–201, 201fLysophosphatidylcholine. See LysolecithinLysophospholipase, 200, 201fLysophospholipids, 116, 116fLysosomal degradation pathway, defect inin lipidoses, 203Lysosomal enzymes, 623in I-cell disease, 431, 432t, 531–532,532fLysosomal hydrolases, deficiencies of,532–533, 533tLysosomesin oligosaccharide processing, 524protein entry into, 507, 507f, 508tdisorders associated with defects in,512t, 513Lysozyme, 621tLysyl hydroxylasediseases caused by deficiency of, 538tin hydroxylysine synthesis, 240, 537Lysyl oxidase, 537, 539Lytic pathway, 379, 379fD-Lyxose, 104f, 105tMac-1, 529, 529tα 2 -Macroglobulin, 583t, 590, 624antithrombin activity of, 603Macromolecules, cellular transport of,428–431, 429f, 430fMad cow disease (bovine spongiformencephalopathy), 37Magnesium, 496tin chlorophyll, 270in extracellular and intracellular fluid,416, 416tMajor groove, in DNA, 305f, 306operon model and, 378Malate, 132f, 133Malate dehydrogenase, 132f, 133Malate shuttle, 99, 100fMALDI. See Matrix-assisted laser-desorptionMaleylacetoacetate, in tyrosine catabolism,254f, 255Malic enzyme, 156t, 157in NADPH production, 176, 176fMalignancy/malignant cells. SeeCancer/cancer cellsMalignant hyperthermia, 556, 564–565,565f, 569tMalonaterespiratory chain affected by, 95, 96fsuccinate dehydrogenase inhibition by,67–68, 67fMalonyl-CoA, in fatty acid synthesis, 173,174fMalonyl transacylase, 173, 174f, 175fMaltase, 475Maltose, 106–107, 107f, 107tMammalian target of rapamycin (mTOR),in insulin signal transmission,466f, 467Mammotropin. See ProlactinManganese, 496tMannosamine, 169, 171fD-Mannosamine, 106Mannose, in glycoproteins, 516tD-Mannose, 104f, 105tα-D-Mannose, 104fMannose-binding protein, deficiency of, 533Mannose 6-phosphate/mannose 6-P signal,526in I-cell disease, 531, 532, 532fin protein flow, 507, 508tMannosidosis, 532–533, 533tMAP (mitogen-activated protein) kinasein insulin signal transmission, 466f, 467in Jak/STAT pathway, 467Maple syrup urine disease (branched-chainketonuria), 259Marasmus, 80, 237, 478, 478–479Marble bone disease (osteopetrosis), 552Marfan syndrome, fibrillin mutationscausing, 539–540, 540fMaroteaux-Lamy syndrome, 546tMass spectrometry, 27, 27fcovalent modifications detected by, 27,27f, 27tfor glycoprotein analysis, 514, 515ttandem, 27transcript-protein profiling and, 412Mast cells, heparin in, 545Matrixextracellular, 535–555. See also specificcomponentmitochondrial, 92, 93f, 130Matrix-assisted laser-desorption (MALDI),in mass spectrometry, 27Matrix-processing peptidase, 499Matrix proteins, 499diseases caused by defects in import of,503Maxam and Gilbert’s method, for DNAsequencing, 404–405Maximal velocity (V max )allosteric effects on, 75–76inhibitors affecting, 68, 68f, 69fMichaelis-Menten equation indetermination of, 65–66, 66fsubstrate concentration and, 64, 64fMcArdle’s disease/syndrome, 152t, 573Mechanically gated ion channels, 568tMediator-related proteins, 472t, 473Medicinepreventive, biochemical researchaffecting, 2relationship of to biochemistry, 1–4, 3fMedium-chain acyl-CoA dehydrogenase,deficiency of, 188Megaloblastic anemiafolate deficiency causing, 482t, 492, 610tvitamin B 12 deficiency causing, 482t,492, 494, 610tMelanocyte-stimulating hormone (MSH),453, 453fMELAS (mitochondrial encephalomyopathywith lactic acidosis andstroke-like episodes), 100–101