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Система RHAG

Антигены Duclos, DSLK и Ola

Статус самостоятельной групповой системы получили антигены, располагающи- есянаRh-ассоциированномгликопротеине(RhAG).РанееRhAGчислилсявсистеме резускакRh50,новскоресталоизвестно,чтосинтезуказанногогликопротеинанаходится под контролем гена, расположенного на 6-й хромосоме независимо от локуса RH,располагающегосяна1-йхромосоме(Daniels[1]).Системавключает3антигена: 2частовстречающихся(DuclosиDSLK)и1редковстречающийся–Ol a.

Антиген Duclos описан Habibi и соавт. в 1978 г. [2]. Вначале он был включен в систему резус под номером Rh38, однако позднее было установлено, что этот антиген является продуктом гена, независимого от локуса RH.

Антиген Ol a (от фамилии семьи Oldeide), обнаруженный Kornstad [3] у норвежцев, также был отнесен с системе резус, поскольку был ассоциирован со

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слабой экспрессией антигенов С, с, Е и е, однако было показано, что он не является частью системы Rh.

Проведено секвенирование ДНК Duclos-отрицательного, DSLK-отрицательного и2Ol a-положительныхлиц.

Рекомбинантные протеины удалось экспрессировать в клетках линии HEK 293. Ранее на этих клетках был экспрессирован мутантный RhAG с аминокислотной заменой Gln 106 Glu , который не реагировал с антителами анти-Duclos. Duclos-отрицательный и DSLK-отрицательный доноры оказались гомозиготными по мутациям Gln 106 Glu и Lys 164 Gln соответственно. Антиген, открываемый антителами анти-DSLK, напоминал антиген Duclos, поэтому получил свое обозначение в виде аббревиатуры DSLK (Duclos-like – Даклос-подобный). Анти-DSLK-антитела, так же как и анти-Duclos-антитела, давали отрицательные реакции с эритроцитами RhnullU–, но реагировали с эритроцитами RhnullU +. Однако анти-DSLK-антитела образовались у Duclos-положительного лица, поэтому не могли быть идентичны анти-Duclos-антителам (Tsuneyama и соавт. [6]).

Два члена норвежской семьи Олдейд, имевшие фенотип Ol(a + ), оказались гетерозиготными по мутации Ser 227 Leu. Один индивид японского происхождения с фенотипом Rhmod был гомозиготным по этой мутации, и его эритроци-

ты также были Ol(a + ) (Tsuneyama и соавт. [6]).

Синтез всех трех антигенов (Duclos, DSLK и Ol a) контролируется одним и тем же геном RHAG, который находится на хромосоме 6 в позиции 6p11-21.1. В номенклатуре ISBT антигены Duclos, Ol a и DSLK получили обозначения 030001, 030002

и030003(RHAG1,RHAG2иRHAG3)соответственно(Tilleyисоавт.[4,5]).

Список литературы

1.DanielsG.L.HumanBloodGroups.–2-nded.–Oxford:BlackwellScience,2002.–560 p.

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4.Tilley L., Gaskell A., Poole J., Daniels G. Duclos-negative and susbstitutions in external loopsoftheRh-associatedglycoprotein//VoxSang.–2008.–V.95(Suppl.).–37(Abstract).

5.Tilley L., Green C., Poole J. et al. A new blood group system, RHAG: three antigens resulting from amino acid substitutions in the Rh-associated glycoprotein // Vox Sang. – 2010. – V. 98. – P. 151–159.

6.Tsuneyama H., Osagawara K., Uchikawa M. et al. Identification of two new mutations in

the RhAG gene of Japanese with Rhmod phenotype // Trasfusion. – 2008. – V. 48 (Suppl.). – P.185A −186A.

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