Molekulare Charakterisierung der Ichthyosen

Ichthyoses comprise an etiologically heterogeneous group of Mendelian disorders of cornification (MEDOC) that affect the entire integument and are characterized by hyperkeratosis and/or visible scaling. In recent years basic research has elucidated the genetic basis of almost all ichthyosis forms and enormously improved diagnostic facilities. The Network for Ichthyoses and Related Keratinization Disorders (NIRK; www.netzwerk-ichthyose.de) and the patient support organization Selbsthilfe Ichthyose e. V. (www.ichthyose.de) provide helpful contact points for physicians and patients in Germany. The first worldwide Ichthyosis Consensus Classification was approved in August 2009. Its nosology is based on clinical presentation but also reflects pathogenic aspects. The major criterion is the distinction between syndromic and non-syndromic forms. Controversial disease names have been re-defined: Ichthyoses due to keratin mutations are referred to under the new umbrella term keratinopathic ichthyosis (KPI), which comprises epidermolytic ichthyosis (mutation in keratin 1 or 10) and superficial epidermolytic ichthyosis (keratin 2). The term autosomal recessive congenital ichthyosis (ARCI) represents the umbrella for harlequin ichthyosis and for the group of lamellar ichthyosis and congenital ichthyosiform erythroderma. The international classification should serve as a reference for future research into these diseases, including further genotype-phenotype correlation studies.     

Nutritive value of thermoammoniated and steam-treated maize stover. I. Intake,digestibility and nitrogen retention

Chopped maize stover, ammoniated at ambient and elevated temperatures or steamed, was evaluated with eight wether lambs in a 4 × 4 double Latin square design. Treatments were: (i) control, 60% H₂O, ensiled for at least 40 days; (ii) 3% NH₃, 60% H₂O for 30 days at 21°C; (iii) 3% NH₃, 60% H₂O for 12 h at 90°C; (iv) steamed at 16.2 kg/cm² and 213°C for 4 min. Ammoniated materials were aerated prior to feeding. All rations were equally supplemented with molasses and soya bean meal while urea was added to treatments (i) and (iv) to increase the crude protein content to at least 12%. Ammoniation at ambient and elevated temperatures increased (P < 0.05) organic matter intake by 30 and 39%, respectively. No difference in intake occurred between the control and steam-treated stovers. However, apparent digestibility of dry matter, organic matter, neutral detergent fibre, acid detergent fibre and cellulose increased (P < 0.05) with all treated stovers. Thermoammoniation produced the greatest (P < 0.05) improvements in digestibility. Improvements in nitrogen retention were slight and non-significant with ammoniated stovers.Thermoammoniation of maize stover with 3% NH₃ and 60% H₂O for 12 h at 90°C increased the extent of digestion over ambient ammoniation and steam treatment, and is a rapid and effective method of improving the feeding value of low quality roughages.     

A Nitrate Reductase-Inactivating Factor from Barley (Hordeum distichum L.) Leaves

A nitrate reductase-inactivating factor (NR-IAF) was detectedin a crude extract from 8-day-old barley (Hordeum distichumL. cv. Daisen-gold) leaves by chromatofocusing. The factor seemedto be a proteolytic enzyme with a cysteine residue at its activesite because 1) it was thermo-labile, and trypsin treatmentcaused loss of activity; 2) p-chloromercuribenzoic acid andiodoacetamide inhibited its activity; 3) leupeptin, an inhibitorof trypsin-like enzymes, also inhibited its activity; and 4)proteolytic activity toward azocasein was detected for the factorpreparation. The factor did not affect the activities of nitritereductase, glutamate dehydrogenase and xanthine oxidase. (Received March 22, 1983; Accepted July 30, 1983)