HEMPAS

HEMPAS (MIM 224100) Abnormalities in certain enzymes (eg, mannosidase II and others) involved in the biosynthesis of N-glycans, particularly affecting the red blood cell membrane. 

Sram, R.J., Benes, V. Zudova, Z. (1970) Induction of dominant lethals in mice by TEPA and HEMPA. Eolia biol. (Praha), 16, 407-415... 

Fukuda, M. N Hempas disease Genetic defect of glycosyla-tion. Glycobiology 1 9-15, 1990. 

SYNS EASTMAN INHIBITOR HPT ENT 50,882 HEMPA HEXAMETAPOL HEXAMETHYLPHOS-PHORIC ACID TRIAMIDE (MAK) HEXAMETHYL-PHOSPHORIC TRIAMIDE N,N,N,N -HEXA-... 

Fukuda MN. Congenital dyserythropoietic anaemia type II (HEMPAS) and its molecular basis. Baillieres Clin Haematol 1993 6 493-511. 

Fukuda MN. HEMPAS. Hereditary erythroblastic multinuclearity with positive acidified serum lysis test. Biochim Biophys Acta 1999 1455 231-239. 

GNT-II HEMPAS Substantially reduced activity of GNT-II responsible for ineffective erythropoiesis, multinucleated erythroblasts, anemia... 

HEMPAS hereditary erythroblastic multinuclearity with positive acidihed-serum lysis test... 

FIGURE 7. Survival of diploid progeny from habrobracon males after topical treatments with chemosterilants. O = 9.1 % Tretamine [2,4,6-tris(l-aziridinyl)-5-triazine]. = 0.1 % Metepa [tris(2-methyl-l-aziridinyl)phosphine oxide]. A = 0.1 % HEMPA (hexamethylphosphoric triamide). A = 0.01 % P,P-bis(l-aziridinyl)-, iV-dimethylphosphoric amide. % — 0.001 % TEPA [tris(l-aziridinylphosphine oxide)]. Data from LaChance and Leverich (1968). 

FIGURE 5. Tepa and related compounds (A) tris(l-aziridinyl)phosphine oxide (tepa) B) dimethylamino-bis(l-aziridinyl)phosphine oxide (C) bis(dimethylamino)-l-aziridinylphosphine oxide (D) hexamethylphosphoric triamide or tris(dimethyl-amino)phosphine oxide (hempa). 

The effect of hempa has previously been studied in human leukoc)rtes in vitro by Chang and Klassen (1968) and in plant roots by Ninan and Wilson... 

In these studies, as well as in those by Sturelid and Hartley (Table 4), hempa was found to be inactive, or practically so. However, in spite of its lack of a chromosome-breaking activity, hempa has been found to be mutagenic in Bracon hehetor (Palmquist and LaChance, 1966) and in Drosophila melanogaster (Ninan and Wilson, 1969). 

Hexamethyl-3-phenyl-3-[(trimethylsilyl) oxy] trisiloxane. See Phenyl trimethicone Hexamethylphosphoramide Hexamethylphosphoric triamide. See Hempa Hexamethyl p-rosaniline chloride Hexamethyl p-rosaniline hydrochloride. See Basic violet 3 Hexamethylsilazane. See Hexamethyidisilazane... 

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