mandibuloacral dysplasia with type-B lipodystrophy

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mandibuloacral dysplasia with type-B lipodystrophy

A rare autosomal recessive disorder (OMIM:608612) characterised by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of cranial suture, joint contractures and type-B lipodystrophy, which is characterised by a generalised loss of fat. 

Molecular pathology
Caused by defects of ZMPSTE24, which encodes a zinc metalloproteinase involved in the post-translational proteolytic cleavage of farnesylated prelamin A to form mature lamin A.
References in periodicals archive ?
Interestingly, the assembly of vimentin microfibrils in MADB fibroblasts improved with rapamycin and dimethylsulfoxide.
We conclude that rapamycin and dimethylsulfoxide are beneficial for improving nuclear morphology and cell proliferation of MADB fibroblasts.
As mentioned in Section 2, the important aim of the stability analysis of delayed GRNs is to determine the MADBs. And the stability criterion that provides bigger MADBs is less conservative than the one that gives smaller ones.
Assume that [[sigma].sub.1] = 0.1, [[sigma].sub.2] = 0.3, [[sigma].sub.d] = 0.7, and [[tau].sub.d] = 1.5 [19], and the MADBs of [[tau].sub.2] with respect to various [[tau].sub.1] obtained by the proposed criteria are given in Table 1, where the MADBs reported in the literature are also listed for comparison.
The MADB is, in many ways, in rather a worse condition than its troubled state-owned siblings.
Under the Thein Sein administration, the MADB has lifted the maximum credit it can advance to cultivators to K100,000 per acre (up to a maximum of K500,000 for holdings of 10 acres or more).
For given [d.sub.m], [alpha], [beta], and [d.sub.d], utilizing Theorem 4, we calculate the MADB [d.sub.M] which can guarantee the stability of system (1).
Supply and update of data from 1.1.2017 to 31.12.2018 concerning the import duties and internal taxes applicable in the Democratic Republic of the Congo for the Tariffs section of the market access database (MADB).
Table 1: The maximum allowable delay bound (MADB) [h.sub.2] for different values of [gamma].
Supply and update of data for the MADB, for the period 2016 through 2018 concerning the following:
Supply and update of data for the MADB, for the period 2016 through 2018 concerning the following: import duties and taxes applicable to a minimum of 108 designated customs areas to be part of the ~Tariffs~ section of the MADB which will also include information on ~Preferential rules of origin~ applicable to countries with which the EU has concluded an agreement, import procedures and formalities applicable to a minimum of 108 designated customs areas to be included in the ~Procedures and formalities~ section of the MADB.Estimated cost excluding VAT: 5 832 000 EUR
Supply and update of data, for the years 2014 and 2015, concerning the import procedures and formalities applicable in 70 customs areas, to be included in the ~Procedures and formalities~ section of the market access database (MADB).