0 9 Decreased decrease VBN 10 23 concentration concentration NN 24 26 of of IN 27 48 1,25-dihydroxyvitamin 1,25-dihydroxyvitamin NN 49 51 D3 d3 NN 52 61 receptors receptor NNS 62 64 in in IN 65 75 peripheral peripheral JJ 76 87 mononuclear mononuclear JJ 88 93 cells cell NNS 94 96 of of IN 97 105 patients patient NNS 106 110 with with IN 111 119 X-linked x-linked JJ 120 136 hypophosphatemic hypophosphatemic JJ 137 144 rickets ricket NNS 144 145 : : : 146 152 effect effect NN 153 155 of of IN 156 165 phosphate phosphate NN 166 181 supplementation supplementation NN 181 182 . . . 184 192 Abnormal abnormal JJ 193 198 renal renal JJ 199 206 tubular tubular JJ 207 216 phosphate phosphate NN 217 226 transport transport NN 227 229 is be VBZ 230 240 considered consider VBN 241 243 to to TO 244 246 be be VB 247 250 the the DT 251 258 primary primary JJ 259 265 defect defect NN 266 268 in in IN 269 277 X-linked x-linked JJ 278 294 hypophosphatemic hypophosphatemic JJ 295 302 rickets ricket NNS 303 304 ( ( ( 304 307 XLH XLH NNP 307 308 ) ) ) 308 309 . . . 310 317 However however RB 317 318 , , , 319 322 the the DT 323 333 resistance resistance NN 334 336 to to TO 337 344 vitamin vitamin NN 345 346 D D NNP 347 356 treatment treatment NN 357 359 in in IN 360 363 XLH XLH NNP 364 370 cannot cannot MD 371 373 be be VB 374 383 explained explain VBN 384 386 by by IN 387 403 hypophosphatemia hypophosphatemia NN 404 409 alone alone RB 409 410 . . . 411 416 Since since IN 417 421 most most JJS 422 424 of of IN 425 428 the the DT 429 436 actions action NNS 437 439 of of IN 440 447 vitamin vitamin NN 448 449 D D NNP 450 453 are be VBP 454 462 mediated mediate VBN 463 465 by by IN 466 469 its its PRP$ 470 479 receptors receptor NNS 480 481 ( ( ( 481 484 VDR VDR NNP 484 485 ) ) ) 485 486 , , , 487 500 abnormalities abnormality NNS 501 503 of of IN 504 507 VDR VDR NNP 508 512 have have VBP 513 517 been be VBN 518 528 postulated postulate VBN 529 531 in in IN 532 535 XLH XLH NNP 535 536 . . . 537 539 In in IN 540 545 order order NN 546 548 to to TO 549 560 investigate investigate VB 561 565 this this DT 566 577 possibility possibility NN 577 578 , , , 579 581 we we PRP 582 590 measured measure VBD 591 594 the the DT 595 608 concentration concentration NN 609 611 of of IN 612 615 VDR VDR NNP 616 618 in in IN 619 632 PHA-activated pha-activated JJ 633 643 peripheral peripheral JJ 644 655 mononuclear mononuclear JJ 656 661 cells cell NNS 662 666 from from IN 667 669 10 10 CD 670 673 XLH XLH NNP 674 682 patients patient NNS 682 683 . . . 684 692 Patients patient NNS 693 700 without without IN 701 710 phosphate phosphate NN 711 726 supplementation supplementation NN 727 733 showed show VBD 734 747 significantly significantly RB 748 753 lower low JJR 754 767 concentration concentration NN 768 769 ( ( ( 769 773 21.7 21.7 CD 774 777 +/- +/- SYM 778 781 5.1 5.1 CD 782 789 fmol/mg fmol/mg NN 790 797 protein protein NN 797 798 , , , 799 803 mean mean NN 804 807 +/- +/- SYM 808 811 SEM sem NN 811 812 ) ) ) 813 821 compared compare VBN 822 824 to to TO 825 828 the the DT 829 835 normal normal JJ 836 844 controls control NNS 845 846 ( ( ( 846 850 60.7 60.7 CD 851 854 +/- +/- CC 855 858 4.0 4.0 CD 858 859 ) ) ) 859 860 . . . 861 863 On on IN 864 867 the the DT 868 876 contrary contrary NN 876 877 , , , 878 883 there there EX 884 887 was be VBD 888 890 no no DT 891 902 significant significant JJ 903 913 difference difference NN 914 921 between between IN 922 925 the the DT 926 948 phosphate-supplemented phosphate-supplemented JJ 949 957 patients patient NNS 958 959 ( ( ( 959 963 58.3 58.3 CD 964 967 +/- +/- CC 968 971 2.7 2.7 CD 971 972 ) ) ) 973 976 and and CC 977 985 controls control NNS 985 986 . . . 987 992 There there EX 993 996 was be VBD 997 998 a a DT 999 1010 significant significant JJ 1011 1019 positive positive JJ 1020 1031 correlation correlation NN 1032 1039 between between IN 1040 1043 VDR VDR NNP 1044 1057 concentration concentration NN 1058 1061 and and CC 1062 1067 serum serum NN 1068 1077 phosphate phosphate NN 1078 1079 ( ( ( 1079 1080 P p NN 1081 1085 less less JJR 1086 1090 than than IN 1091 1095 0.05 0.05 CD 1095 1096 ) ) ) 1096 1097 . . . 1098 1100 In in IN 1101 1104 two two CD 1105 1113 patients patient NNS 1113 1114 , , , 1115 1118 VDR VDR NNP 1119 1122 was be VBD 1123 1132 increased increase VBN 1133 1138 after after IN 1139 1144 daily daily JJ 1145 1154 phosphate phosphate NN 1155 1170 supplementation supplementation NN 1171 1174 was be VBD 1175 1182 started start VBN 1182 1183 . . . 1184 1189 These these DT 1190 1197 results result NNS 1198 1206 indicate indicate VBP 1207 1211 that that IN 1212 1213 a a DT 1214 1223 decreased decrease VBN 1224 1237 concentration concentration NN 1238 1240 of of IN 1241 1244 VDR VDR NNP 1245 1254 secondary secondary JJ 1255 1257 to to TO 1258 1268 persistent persistent JJ 1269 1285 hypophosphatemia hypophosphatemia NN 1286 1288 is be VBZ 1289 1292 one one CD 1293 1295 of of IN 1296 1299 the the DT 1300 1306 causes cause NNS 1307 1309 of of IN 1310 1317 vitamin vitamin NN 1318 1319 D D NNP 1320 1330 resistance resistance NN 1331 1333 in in IN 1334 1337 XLH XLH NNP 1337 1338 . . .