This paper reviews the traditional practice of Klila production, its microbiological, physicochemical and nutritional properties and aims to raise awareness of Klila. The main objective of this review is to highlight the typicality of Klila and to propose a form of labelling that could help promote it. The Klila is a traditional extra hard cheese from southern Algeria. Nomadic tribes produce it from goat, sheep, or cow milk. Curdled milk is obtained by spontaneous fermentation. It is churned to recover the butter. The co-product, a sour, fat-free milk: Lben, undergoes a moderate heat treatment to get a fresh cheese which, after a dehydration process, gives the Klila. The chemical composition of this product varies regarding fat content. It has a very low water content (less than 10%) and comes in very hard pieces of varying size and irregular shape. The dry matter, for instance, can vary from 33 to 95%. Water availability (Aw) varies from 0.32 to 0.467. Fat rate can vary a lot from region to region from 9.5 to 29.3 g/100 g of cheese. The protein rate also varies a lot: between 29.9 and 71.4 g/100 g of cheese. The lactose concentration can range within 1.2 to 2.4 g/100 mL. The microbiological quality of Klila depends mainly on the quality of the raw milk used and all hygienic factors that surround milk production. The physicochemical conditions that prevail inside Klila are not favourable for the microbial growth. The Klila is an example of adaptation of the nomads’ lifestyle in the Algerian Sahara who use it as a culinary adjunct for nutritional and therapeutic purposes. The Klila can be grinded to be put as ingredient in “Zrizri” dessert with dates and clarified butter (smen). Some people prefer to consume fresh Klila with dates and green tea.
Atypical progeroid syndromes (APS) are premature aging syndromes caused by pathogenic LMNA missense variants, associated with unaltered expression levels of lamins A and C, without accumulation of wild‐type or deleted prelamin A isoforms, as observed in Hutchinson‐Gilford progeria syndrome (HGPS) or HGPS‐like syndromes. A specific LMNA missense variant, (p.Thr528Met), was previously identified in a compound heterozygous state in patients affected by APS and severe familial partial lipodystrophy, whereas heterozygosity was recently identified in patients affected by Type 2 familial partial lipodystrophy. Here, we report four unrelated boys harboring homozygosity for the p.Thr528Met, variant who presented with strikingly homogeneous APS clinical features, including osteolysis of mandibles, distal clavicles and phalanges, congenital muscular dystrophy with elevated creatine kinase levels, and major skeletal deformities. Immunofluorescence analyses of patient‐derived primary fibroblasts showed a high percentage of dysmorphic nuclei with nuclear blebs and typical honeycomb patterns devoid of lamin B1. Interestingly, in some protrusions emerin or LAP2α formed aberrant aggregates, suggesting pathophysiology‐associated clues. These four cases further confirm that a specific LMNA variant can lead to the development of strikingly homogeneous clinical phenotypes, in these particular cases a premature aging phenotype with major musculoskeletal involvement linked to the homozygous p.Thr528Met variant.
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