Ibn al-ʻArabī and Abū Yazīd al-Bistāmī

Many Sufis appear in Ibn al-‛Arabī’s al-Futūhāt al-Makkiyya, a treatise in which the author puts forward his principal ideas. Abū Yazīd al-Bistāmī is mentioned in this work 143 times, more than any other Sufi. The present article seeks to examine Ibn al-‛Arabī’s attitude toward his Sufi predecessor by considering al-Bistāmi’s personality as it appears in Ibn al-‛Arabī, as well as Ibn al-‛Arabī’s views on al-Bistāmī’s Sufi practice, his presence as a model of moral conduct and his mystical philosophical notions. Ibn al-‛Arabī does not accept all the notions and statements expressed by al-Bistāmī. Sometimes he mitigates al-Bistāmi’s daring assertions and thus reveals his unfavorable attitude toward shatahāt. However, in some cases al-Bistāmī’s statements serve as corroboration for Ibn al-‛Arabī’s theses.Aparecen muchos sufíes en la obra de Ibn al-‛Arabī al-Futūhāt al-Makkiyya, un tratado en el que el autor presenta sus ideas principales. En esta obra se menciona a Abū Yazīd al-Bistāmi un total de 143 veces, más que a cualquier otro sufí. Este artículo tiene el propósito de examinar la actitud de Ibn al-‛Arabī hacia su predecesor sufí considerando la personalidad de al-Bistāmī tal y como aparece en la obra de Ibn al-‛Arabī, además de analizar las ideas de Ibn al-‛Arabī sobre las prácticas al-Futūhāt sufíes de al-Bistāmī, su presencia como modelo de conducta moral y sus ideas filosóficas místicas. Ibn al-‛Arabī no acepta todas las ideas y declaraciones de al-Bistāmi. A veces suaviza sus afirmaciones más atrevidas y de esta manera revela su actitud negativa hacia šatahāt. Sin embargo, en algunos casos Ibn al-‛Arabī se sirve de las afirmaciones de al-Bistāmi para corroborar sus tesis

Objectives and Methods of Iron Chelation Therapy

Recent developments in the understanding of the molecular control of iron homeostasis provided novel insights into the mechanisms responsible for normal iron balance. However in chronic anemias associated with iron overload, such mechanisms are no longer sufficient to offer protection from iron toxicity, and iron chelating therapy is the only method available for preventing early death caused mainly by myocardial and hepatic damage. Today, long-term deferoxamine (DFO) therapy is an integral part of the management of thalassemia and other transfusion-dependent anemias, with a major impact on well-being and survival. However, the high cost and rigorous requirements of DFO therapy, and the significant toxicity of deferiprone underline the need for the continued development of new and improved orally effective iron chelators. Within recent years more than one thousand candidate compounds have been screened in animal models. The most outstanding of these compounds include deferiprone (L1); pyridoxal isonicotinoyl hydrazone (PIH) and; bishydroxy- phenyl thiazole. Deferiprone has been used extensively as a substitute for DFO in clinical trials involving hundreds of patients. However, L1 treatment alone fails to achieve a negative iron balance in a substantial proportion of subjects. Deferiprone is less effective than DFO and its potential hepatotoxicity is an issue of current controversy. A new orally effective iron chelator should not necessarily be regarded as one displacing the presently accepted and highly effective parenteral drug DFO. Rather, it could be employed to extend the scope of iron chelating strategies in a manner analogous with the combined use of medications in the management of other conditions such as hypertension or diabetes. Coadministration or alternating use of DFO and a suitable oral chelator may allow a decrease in dosage of both drugs and improve compliance by decreasing the demand on tedious parenteral drug administration. Combined use of DFO and L1 has already been shown to result in successful depletion of iron stores in patients previously failing to respond to single drug therapy, and to lead to improved compliance with treatment. It may also result in a “shuttle effect” between weak intracellular chelators and powerful extracellular chelators or exploit the entero-hepatic cycle to promote fecal iron excretion. All of these innovative ways of chelator usage are now awaiting evaluation in experimental models and in the clinical setting

Dental profile of patients with Gaucher disease

BACKGROUND: This study was conducted to determine whether patients with Gaucher disease had significant dental pathology because of abnormal bone structure, pancytopenia, and coagulation abnormalities. METHODS: Each patient received a complete oral and periodontal examination in addition to a routine hematological evaluation. RESULTS: Gaucher patients had significantly fewer carious lesions than otherwise healthy carriers. Despite prevalence of anemia, there was no increase in gingival disease; despite the high incidence of thrombocytopenia, gingival bleeding was not noted; and despite radiological evidence of bone involvement, there was no greater incidence loss of teeth or clinical tooth mobility. CONCLUSIONS: These data represent the first survey of the oral health of a large cohort of patients with Gaucher disease. It is a pilot study of a unique population and the results of the investigation are indications for further research. Based on our findings, we recommend regular oral examinations with appropriate dental treatment for patients with Gaucher disease as for other individuals. Consultation between the dentist and physician, preferably one with experience with Gaucher disease, should be considered when surgical procedures are planned

TFEB regulates lysosomal proteostasis

Loss-of-function diseases are often caused by destabilizing mutations that lead to protein misfolding and degradation. Modulating the innate protein homeostasis (proteostasis) capacity may lead to rescue of native folding of the mutated variants, thereby ameliorating the disease phenotype. In lysosomal storage disorders (LSDs), a number of highly prevalent alleles have missense mutations that do not impair the enzyme's catalytic activity but destabilize its native structure, resulting in the degradation of the misfolded protein. Enhancing the cellular folding capacity enables rescuing the native, biologically functional structure of these unstable mutated enzymes. However, proteostasis modulators specific for the lysosomal system are currently unknown. Here, we investigate the role of the transcription factor EB (TFEB), a master regulator of lysosomal biogenesis and function, in modulating lysosomal proteostasis in LSDs. We show that TFEB activation results in enhanced folding, trafficking and lysosomal activity of a severely destabilized glucocerebrosidase (GC) variant associated with the development of Gaucher disease (GD), the most common LSD. TFEB specifically induces the expression of GC and of key genes involved in folding and lysosomal trafficking, thereby enhancing both the pool of mutated enzyme and its processing through the secretory pathway. TFEB activation also rescues the activity of a β-hexosaminidase mutant associated with the development of another LSD, Tay–Sachs disease, thus suggesting general applicability of TFEB-mediated proteostasis modulation to rescue destabilizing mutations in LSDs. In summary, our findings identify TFEB as a specific regulator of lysosomal proteostasis and suggest that TFEB may be used as a therapeutic target to rescue enzyme homeostasis in LSDs

Ibn al-ʻArabī y Abū Yazīd al-Bistāmī

Many Sufis appear in Ibn al-‛Arabī’s al-Futūhāt al-Makkiyya, a treatise in which the author puts forward his principal ideas. Abū Yazīd al-Bistāmī is mentioned in this work 143 times, more than any other Sufi. The present article seeks to examine Ibn al-‛Arabī’s attitude toward his Sufi predecessor by considering al-Bistāmi’s personality as it appears in Ibn al-‛Arabī, as well as Ibn al-‛Arabī’s views on al-Bistāmī’s Sufi practice, his presence as a model of moral conduct and his mystical philosophical notions. Ibn al-‛Arabī does not accept all the notions and statements expressed by al-Bistāmī. Sometimes he mitigates al-Bistāmi’s daring assertions and thus reveals his unfavorable attitude toward shatahāt. However, in some cases al-Bistāmī’s statements serve as corroboration for Ibn al-‛Arabī’s theses.Aparecen muchos sufíes en la obra de Ibn al-‛Arabī al-Futūhāt al-Makkiyya, un tratado en el que el autor presenta sus ideas principales. En esta obra se menciona a Abū Yazīd al-Bistāmi un total de 143 veces, más que a cualquier otro sufí. Este artículo tiene el propósito de examinar la actitud de Ibn al-‛Arabī hacia su predecesor sufí considerando la personalidad de al-Bistāmī tal y como aparece en la obra de Ibn al-‛Arabī, además de analizar las ideas de Ibn al-‛Arabī sobre las prácticas al-Futūhāt sufíes de al-Bistāmī, su presencia como modelo de conducta moral y sus ideas filosóficas místicas. Ibn al-‛Arabī no acepta todas las ideas y declaraciones de al-Bistāmi. A veces suaviza sus afirmaciones más atrevidas y de esta manera revela su actitud negativa hacia šatahāt. Sin embargo, en algunos casos Ibn al-‛Arabī se sirve de las afirmaciones de al-Bistāmi para corroborar sus tesis

The bi-lâ kayfa doctrine and its foundations in Islamic theology

Bu makalenin orjinali, “The Bi-Lâ Kayfa Doctrine and its Foundations in Islamic Theology”, Arabica: Journal of Arabic and Islamic Studies, XLII/3 (1995), Leiden künyesindeki yayında yayınlanmıştır.İslâm kelâmında, Kur’an ve Sünnet literatüründeki teşbihî ifadeleri açıklamak için üç farklı yol vardır. Bunlardan biri, nasıl olduğunu sormaksızın veya yorum yapmaksızın demek olan bi-lâ keyfe doktrinidir. Bu doktrinin taraftarlarına göre teşbihî ifadeler, keyfiyyetini yorumlamaya kalkmaksızın olduğu gibi kabul edilmelidir. Bu makalede, İbn Hanbel’den, İbn Teymiyye’ye kadar pek çok müslüman alim tarafından, bi-lâ keyfe doktrini lehine geliştirilen çeşitli deliller ortaya konulmaktadır.In Islamic Theology, three methods have been used to explain the anthropomorphic expressions in Quran and the Sunna literature. One of them is bi-lâ kayfa doctrine, which means “without asking how” or “without comment.” As far as the adherents to this doctrine are concerned the anthropomorphic expressions are to be accepted without to interpret them in terms of modality. In this article, the arguments, which are based on scriptural, linguistic, rational and philosophical considerations and developed by several muslim scholars, from Ibn Hanbal to Ibn Taymiyya, in favour of bi-lâ kayfa doctrine are revealed