PMID- 3859465
OWN - NLM
STAT- MEDLINE
DCOM- 19850801
LR  - 20211203
IS  - 0340-6717 (Print)
IS  - 0340-6717 (Linking)
VI  - 70
IP  - 2
DP  - 1985
TI  - The interaction of hemoglobin O Arab with Hb S and beta+ thalassemia among 
      Israeli Arabs.
PG  - 119-25
AB  - We have studied 105 individuals in the village of Jasser El Zarka in the Northern 
      Coast of Israel of whom 59% had at least one abnormal hemoglobin. Of the 
      individuals studied 41% were AA, 13.3% AS, 28.6% AOArab, 10.5% SOArab, 0.9% SS, 
      38% OArab-beta + Thal, and 1.9% beta Thal trait. The SOArab double heterozygotes 
      were characterized by a normal mean corpuscular volume (MCV) and mean corpuscular 
      hemoglobin concentration (MCHC), and an increase of Hb F (11.7 +/- 4.3%) and 
      2,3-diphosphoglycerate levels (27.8 microns/g Hb). The increase of Hb F is higher 
      than the one seen among OArabs of other ethnic backgrounds. Their clinical course 
      was moderately severe and osteoporosis was quite frequent. The interactions of Hb 
      OArab and Hb S were studied in vitro and it was confirmed the Hb OArab lowers the 
      minimal gelling concentration of mixtures with Hb S (as compared to mixtures of 
      Hb S and Hb A), but that this effect is ionic-strength dependent. Our data are in 
      conflict with previous claims that Hb OArab mixtures with Hb S polymerized almost 
      as much as pure S. Oxygen association curves show a significant displacement of 
      the p50 to the right, but the effect of oxygen dissociation is less apparent. The 
      displacement was not nearly as significant as with SS cells, confirming our 
      gelation data. Blood group determinations establish that these Arab populations 
      had black African admixture. The Hb OArab/beta + Thal double heterozygotes 
      exhibit moderate anemia (10.3 g% of Hb) and the percentage of Hb A was 17.2 +/- 
      1.8%. The fetal Hb was 5.4 +/- 2.1% and the 2,3-diphosphoglycerate level in two 
      cases was 17.4 mumol/g Hb. The only case of a homozygote SS had moderate anemia 
      (10.3g Hb%), 25.7% of Hb F, and a very benign course.
FAU - Rachmilewitz, E A
AU  - Rachmilewitz EA
FAU - Tamari, H
AU  - Tamari H
FAU - Liff, F
AU  - Liff F
FAU - Ueda, Y
AU  - Ueda Y
FAU - Nagel, R L
AU  - Nagel RL
LA  - eng
GR  - HL 21016/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - Germany
TA  - Hum Genet
JT  - Human genetics
JID - 7613873
RN  - 0 (Genetic Markers)
RN  - 0 (Hemoglobin, Sickle)
RN  - 0 (Hemoglobins, Abnormal)
RN  - 0 (Isoantigens)
RN  - 9035-17-0 (hemoglobin Arab)
RN  - S88TT14065 (Oxygen)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Erythrocyte Indices
MH  - *Ethnicity
MH  - Female
MH  - Genetic Markers
MH  - Hemoglobin, Sickle/*genetics
MH  - Hemoglobins, Abnormal/*genetics
MH  - Heterozygote
MH  - Humans
MH  - Isoantigens/genetics
MH  - Israel
MH  - Male
MH  - Oxygen/blood
MH  - Pedigree
MH  - Thalassemia/*genetics
EDAT- 1985/01/01 00:00
MHDA- 1985/01/01 00:01
CRDT- 1985/01/01 00:00
PHST- 1985/01/01 00:00 [pubmed]
PHST- 1985/01/01 00:01 [medline]
PHST- 1985/01/01 00:00 [entrez]
AID - 10.1007/BF00273069 [doi]
PST - ppublish
SO  - Hum Genet. 1985;70(2):119-25. doi: 10.1007/BF00273069.