T-cell Large Granular Lymphocytic Leukemia with a STAT3 mutation successfully treated with Cord Blood Transplantation.

A 64-year-old woman presented with agranulocytosis, anemia, and bacteremia, leading to a diagnosis of T-cell large granular lymphocytic leukemia (T-LGLL). A molecular analysis identified a signal transducer and activator of transcription 3 (STAT3) Y640F variant. Initial treatment with cyclophosphamide and prednisolone did not improve her condition, but serious infections were observed.

Feeding induces expression of heat shock proteins that reduce oxidative stress.

Heat shock proteins (Hsps) are induced in response to various kinds of environmental and physiological stresses. However, it is unclear whether Hsps play roles in protecting cells in the digestive organs against xenobiotic chemicals. Here, we found that feeding induces expression of a set of Hsps specifically in the mouse liver and intestine by activating heat shock transcription factor 1 (HSF1).

Heat shock transcription factor HSF1 is required for survival of sensory hair cells against acoustic overexposure.

To analyze the role of heat shock response in the cochleae, we induced major heat shock proteins, Hsp70, Hsp90, and Hsp27 by perfusion of hot saline into the middle ear cavity (called 'local heat shock') in guinea pigs. Hsps were induced in almost all of the cochlear cells including the sensory hair cells in the organ of Corti. We showed that loss of both the sensory hair cells and the auditory function induced by acoustic overexposure was inhibited by pretreatment of the inner ear with local heat shock.

Activation of heat shock genes is not necessary for protection by heat shock transcription factor 1 against cell death due to a single exposure to high temperatures.

Heat shock response, which is characterized by the induction of a set of heat shock proteins, is essential for induced thermotolerance and is regulated by heat shock transcription factors (HSFs). Curiously, HSF1 is essential for heat shock response in mammals, whereas in avian HSF3, an avian-specific factor is required for the burst activation of heat shock genes. Amino acid sequences of chicken HSF1 are highly conserved with human HSF1, but those of HSF3 diverge significantly.