JAHK: a low frequency antigen associated with the rG complex of the Rh blood group system.

We have investigated a 'new' low frequency antigen JAHK, which is a marker for the rare Rh gene complex rG. The rG haplotype does not produce any D, c or E antigens, but does produce a strong G antigen. The rG haplotype [d(C)(e)G] is associated with weak C and weak e antigens. Three unrelated rG/dce individuals and one rG/rG propositus were JAHK+. In addition, three propositi whose red cells had a typical expression of C and/or e antigen, which could not be shown to be rG because of a normal D antigen produced by the haplotype in trans, were also JAHK+. Families of three of the propositi demonstrate the inheritance of JAHK as a Mendelian dominant character. It is likely that the JAHK antigen results from conformational changes in an RhCcEe protein that has the amino acid characteristic of c antigen at position 16 and the amino acid residues characteristic of C antigen at positions 60, 68, and 103. JAHK has been assigned the number RH53.

The role of G protein alpha subunits in the infection process of the gray mold fungus Botrytis cinerea.

To identify signal transduction pathways of the gray mold fungus Botrytis cinerea involved in host infection, we used heterologous hybridization and a polymerase chain reaction (PCR)-based approach to isolate two genes (bcg1 and bcg2) encoding alpha subunits of heterotrimeric GTP-binding proteins. Both genes have homologues in other fungi: bcg1 is a member of the G alpha(i) class, whereas bcg2 has similarities to the magC gene of Magnaporthe grisea and the gna-2 gene of Neurospora crassa. Reverse-transcription (RT)-PCR experiments showed clearly that both genes are expressed at very early stages in infected bean leaves. Gene replacement experiments were performed for both genes. bcg1 null mutants differ in colony morphology from the wild-type strain, do not secrete extracellular proteases, and show clearly reduced pathogenicity on bean and tomato. Conidia germination and penetration of plant tissue is not disturbed in bcg1 mutants, but the infection process stops after formation of primary lesions. In contrast, bcg2 mutants show wild-type colony morphology in axenic culture and are only slightly reduced in pathogenicity. Complementation of bcg1 mutants with the wild-type gene copy led to the full recovery of colony morphology, protease secretion, and pathogenicity on both host plants. Application of exogenous cyclic AMP restored the wild-type growth pattern of bcg1 mutants, but not the protease secretion, implicating an essential role of BCG1 in different signaling pathways.

Frequencies of the blood groups ABO, Rhesus, D category VI, Kell, and of clinically relevant high-frequency antigens in south-western Germany.

BACKGROUND: Current estimates of blood group frequencies in Germany were often derived from studies involving less than 12,000 individuals. The frequency of the D category VI was unknown. METHODS: ABO. Kell, and Rhesus blood group data of more than 600,000 donors were reviewed. Allele frequencies were derived by the maximum-likelihood method. The frequency of D category VI was determined in more than 70,000 Rhesus typings. RESULTS: ABO allele frequencies were: O: 0.640, A: 0.279. B: 0.081. Rhesus haplotype frequencies were: cde: 0.394, CDe: 0.431, cDE: 0.136, cDe: 0.021, and Cde: 0.011. D category VI represented 7%, of all weak D (formerly D(u)). The 95% confidence interval for the D category VI frequency was 1:3,600-1:11,200. Kell allele frequencies were: K: 0.040, and k: 0.960, 95% confidence intervals for rare phenotypes were: Oh: 1:88,000-1:1,760,000, p: 1:200,000-1:5,200,000. Rh(null): 1:180,000-1:10,300,000, and D-deletion: 1: 180,000-0. CONCLUSIONS: We presented refined estimates of ABO, Rhesus D and Kell blood group frequencies and established reliable frequency estimates for Rhesus haplotype and some rare blood groups. The prevalence of D category VI was about 0.02%, which necessitates specific detection for Rh-D-negative transfusion therapy. A protocol is presented for Rh D typing in transfusion recipients. which obviates the need for an antiglobulin test.

[Screening for increased cholesterol values in blood donors]. / Screening auf erhöhte Cholesterinwerte bei Blutspendern.

OBJECTIVE: In a pilot study blood donors were screened on HDL, LDL, and total cholesterol. The practicability and significance of such a screening in a blood bank should be tested. DESIGN: For this purpose, current methods to determine HDL, LDL, and total cholesterol were adapted on microtiter plates and validated. In a period of about 4 weeks all blood donors were tested for the three cholesterol parameters. SETTING: Red Cross blood bank in Baden-Baden, Germany with about 150,000 donations per year. PARTICIPANTS: The HDL, LDL, and total cholesterol results of 10,006 blood donors could be evaluated. INTERVENTIONS: None. RESULTS: The mean total cholesterol was 192 +/- 36 mg/dl, the mean HDL cholesterol 56 +/- 15 mg/dl, and a mean LDL cholesterol of 123 +/- 39 mg/dl was found. The known age-dependency of the measured cholesterol fractions could be confirmed in our blood donor population. According to the current recommendations, more than 20% of the tested blood donors need a therapy. CONCLUSIONS: The study shows that the screening of blood donors for increased total cholesterol for prevention of coronary heart disease could be done with a minimum of costs.

[Monoclonal antibodies in blood group serology. Part 1: Technique of production, ABO and Lewis system]. / Monoklonale Antikörper in der Blutgruppenserologie. Teil 1: Technik der Herstellung, AB0- und Lewis-System.

OBJECTIVE: The introduction of monoclonal antibodies in routine blood group typing has also given rise to new findings. This paper was designed to demonstrate and clarify modifications relevant in practice. SOURCES: Our own experience in the production and application of monoclonal antibodies as well as the relevant literature are taken into consideration. RESULTS: The development and production of murine and human monoclonal antibodies is described especially regarding the selection and the safety of clones. An update of the availability and use of monoclonal antibodies in respect to the different blood group systems is given, with special reference to new findings like the A1(B) and B(A) phenomena. CONCLUSION: The application of monoclonal antibodies in blood group serology has improved the detection of weak variants and the availability of these reagents in general. Further improvements in this technology will make monoclonal antibodies also available for other blood group systems.

[Monoclonal antibodies in blood group serology. Part 2: MNS, Rh and other blood group systems]. / Monoklonale Antikörper in der Blutgruppenserologie. Teil 2: MNS-, Rh- und übrige Systeme.

OBJECTIVE: The introduction of monoclonal antibodies in routine blood group typing has also given rise to new findings. This paper was designed to demonstrate and clarify modifications relevant in practice. SOURCES: Our own experience in the production and application of monoclonal antibodies as well as the relevant literature are taken into consideration. RESULTS: The development and production of murine and human monoclonal antibodies is described especially regarding the selection and the safety of clones. An update of availability and use of monoclonal antibodies in respect to the different blood group systems is given, with special reference to the reactivity of these new reagents with weak D-positive (Du) red blood cells. CONCLUSION: The application of monoclonal antibodies in blood group serology has improved the detection of weak variants and the availability of these reagents in general. Further improvements in this technology will make monoclonal antibodies also available for other blood group systems.

Results of the DGTI workshop on the evaluation of the reactivity of monoclonal anti-D.

All D+ samples were detected by all monoclonal antibodies without problems. Very few of the 3000 D- samples showed a +/- to ++ reaction with some of the IgG type mabs using the Coombs technique. It is still open whether these are specific reactions with weak positive samples, which were negative with polyclonal sera. IgM type mabs detected up to 100% of the Du samples dependent on the Rh-phenotype and the technique used (CCDuee greater than CcDuee greater than ccDuEe). Most of the CCDuee samples reacted very strong and should no longer be regarded as Du.

Results of the DGTI workshop on the evaluation of the reactivity of monoclonal anti-D.

All D+ samples were detected by all monoclonal antibodies (mabs) without any problem. Very few of the 3,000 D- samples showed a +/- to ++ reaction with some of the IgG type mabs using the Coombs technique. It is still open whether these are specific reactions with weakly positive samples, which were negative with polyclonal sera. IgM type mabs detected up to 100% of the Du samples dependent on the Rh phenotype and the technique used (CCDuee > CcDuee > ccDuEe). Most of the CCDuee samples reacted very strongly and should no longer be regarded as Du.

New mutation versus exclusion at the alpha-1-antitrypsin locus: a multifaceted approach in a problematical paternity case.

In a case of disputed paternity with overwhelming indications of fatherhood for the putative father, as supported by serological tests and biostatistical evaluation, a classical exclusion constellation was found at the alpha 1-antitrypsin (PI) locus: mother PI M1; child PI M1M3, and putative father PI M1M2. Additional studies included PI oligonucleotide phenotyping and DNA fingerprint analysis. Results from the entire data set led us to assume a rare genetic event at the paternal PI locus. Intracistronal crossing-over offered the most parsimonious explanation, and was compatible with the PI gene DNA sequence and the amino acid sequences of the molecule and its allelic forms, as well as with the experimental findings.

[Preliminary results of the DGTI Workshop for evaluating the reactivity of monoclonal anti-D]. / Vorläufige Ergebnisse des DGTI Workshops zur Evaluierung der Reaktivität von monoklonalem Anti-D.

Within a working group of the German transfusion society, a workshop was conducted to evaluate the reactivity of monoclonal anti-D supernatants. During this evaluation 10,000 samples were tested, including 325 Du-samples, with 3-5 IgM type antibodies and 3-4 IgG type antibodies, which were selected to also find 'D-variants'. In general, the reactivity with D+ red blood cells was good, and specific in respect to D negative samples. Some samples have been found to have a different pattern of reactivity than the monoclonal antibodies used. Dependent on the method and on the antibody, in some studies up to 100% of Du samples could be detected by anti-D of the IgM type. Some data indicate that the reaction with Du samples is not dependent on titer. In addition, in separate studies the data show a correlation of reactivity in respect to the Rh-phenotype (CcDuee greater than ccDuEe).

[Rheumatoid arthritis, progressive systemic sclerosis without skin involvement and mixed collagen disease in a family. Clinical description of a mother and her 3 adult children--studies of HLA antigens and review of the literature]. / Rheumatoide Arthritis, progressive systemische Sklerose (PSS) ohne Hautbeteiligung und Mischkollagenose (MCTD) in einer Familie. Klinische Beschreibung einer Mutter und ihrer drei erwachsenen Kinder--Untersuchungen der HLA-Antigene und Literaturübersicht.

We report on four members of a family with rheumatic diseases. Rheumatoid arthritis of an extremely severe and mutilating type developed in a 17-year-old boy (Patient 1). The disease showed some characteristics of juvenile chronic arthritis. 20 years later his sister (Patient 2) was affected by progressive systemic sclerosis (PSS) with arthritis, Raynaud's phenomenon, aperistalsis of the esophagus and pulmonary fibrosis, however without skin involvement. After 2 years rheumatoid arthritis developed in the mother of the family (Patient 3) and another 2 years later the second son (Patient 4) was affected by mixed connective tissue disease (MCTD) with arthritis, Raynaud's phenomenon, aperistalsis of the esophagus and a high titer of antibody to extractable nuclear antigen (ENA). Rheumatoid factor was found in Patient 1, 2 and 3. All members of the family expressed HLA-DR3 in association with HLA-B8. Earlier reports in the medical literature of the familial occurrence of rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus and other collagen diseases, e.g. mixed connective tissue disease, are reviewed, with a discussion of the possible etiologic mechanisms.

[Haptoglobin- and group-specific component-phenotypes in healthy carriers of antibodies to hepatitis B surface antigen (author's transl)]. / Haptoglobin- und Group-specific Component-Merkmale bei nicht erkrankten Antikörperträgern gegen Hepatitis B Surface Antigen.

No significant differences could be observed when the haptoglobine and group-specific component phenotypes of 51 unrelated, healthy carriers of antibodies to Hepatitis B surface antigen were compared with normal controls. These findings are contradictory to the suggestion of Prokop [2].

[Cross reactions of anti-E sera with the Rh antigens E and Ew]. / Kreuzreaktionen von Anti-E-Seren mit den Rh-Antigenen E und Ew

Tests with 12 different trade anti-E sera have proven that half of them contain anti-Ew components. Therefore, according to the results of the tests, it must be concluded that, by immunizations with the antigen E, antibodies with the specificity anti-EEw are often be formed.