Biochemical and Molecular Analysis of the Hb Lepore Boston Washington in a Syrian Homozygous Child

<div>Cation-exchange HPLC of Hb tetramers showing Hb Lepore Boston Washington (LBW) eluting in the HbA<sub>2</sub> window (a). Isoelectric focusing of native Hb tetramers. Lane 1: proband; lane 2: normal adult; lane 3: normal newborn; lanes 4 and 5: newborns homozygous for HbF-Sardinia [A<i>γ</i>75(E19) <svg height="9.48169pt" id="M32" style="vertical-align:-0.2455406pt" version="1.1" viewbox="-0.0498162 -9.23615 52.9688 9.48169" width="52.9688pt" xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink"><g transform="matrix(.013,0,0,-0.013,0,0)"><path d="M303 0V28C221 34 213 39 213 125V525C213 610 221 616 303 622V650H38V622C120 616 128 610 128 525V125C128 40 120 34 38 28V0H303Z" id="g121-71"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="341" vert-adv-y="341"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,4.433,0)"><path d="M238 0V26C174 32 166 38 166 104V712C132 700 70 683 18 677V653C81 647 87 645 87 577V104C87 38 78 32 15 26V0H238Z" id="g121-106"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="253" vert-adv-y="253"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,7.722,0)"><path d="M380 106C343 72 306 56 265 56C195 56 116 112 115 248C235 252 361 262 377 265C396 269 400 277 400 297C400 374 333 449 250 449H249C198 449 144 421 103 376S37 269 37 201C37 88 109 -12 232 -12C263 -12 332 6 395 84L380 106ZM225 412C281 412 315 364 314 312C314 297 308 292 290 292C232 290 176 289 120 289C135 370 180 412 225 412Z" id="g121-99"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="425" vert-adv-y="425"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,16.879,0)"><path d="M901 255C861 290 790 361 716 442L694 427L796 280H69V230H796L694 83L716 68C790 149 861 220 901 255Z" id="g117-148"></path><glyph.data ascent="3473" descent="-2876" horiz-adv-x="954" vert-adv-y="954"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,32.913,0)"><path d="M592 498C586 556 582 629 581 675H560C545 656 537 650 510 650H115C87 650 74 652 60 675H40C38 620 33 555 28 495H57C69 539 77 568 89 585C102 607 122 616 205 616H267V123C267 44 259 34 168 28V0H454V28C360 34 352 44 352 123V616H422C498 616 514 608 531 584C542 568 552 543 562 495L592 498Z" id="g121-82"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="617" vert-adv-y="617"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,40.934,0)"><path d="M513 0V26C455 31 447 37 447 103V278C447 398 390 449 309 449C255 449 202 412 166 376V712C127 700 67 684 19 677V653C85 647 87 643 87 580V103C87 37 79 31 19 26L18 0H231V26C171 32 166 39 166 103V341C194 373 232 392 270 392C337 392 368 351 368 269V103C368 38 360 32 302 26V0H513Z" id="g121-102"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="534" vert-adv-y="534"></glyph.data></g><g transform="matrix(.013,0,0,-0.013,47.876,0)"><path d="M181 342V451C133 431 89 419 40 411V388C98 381 102 377 102 311V104C102 38 95 32 33 26V0H263V26C186 32 181 38 181 104V287C203 343 235 372 261 372C277 372 289 366 304 352C310 346 318 345 330 350C349 359 362 379 362 399C362 422 338 449 304 449C256 449 213 393 183 342H181Z" id="g121-112"></path><glyph.data ascent="989" descent="-360" horiz-adv-x="371" vert-adv-y="371"></glyph.data></g></svg>; HBG1: c.227T&gt;C] (b). For Hb F-Sardinia, see [<a href="/journals/bmri/2017/1261972/#B18">16</a>].</div>

BioMed Research International

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Figure 1

Figure 1: Biochemical and Molecular Analysis of the Hb Lepore Boston Washington in a Syrian Homozygous Child 