Askanas V, Engel W K, Bethlem J
Acta Neuropathol. 1978 Jun 30;42(3):247-50. doi: 10.1007/BF00690365.
Leptomeres, the laminated structures consisting of bundles of very fine filaments separated into bands about 260 nm wide by periodic transverse dense lines 20--80 nm wide, were observed frequently in cultured muscle fibers of 8 patients with acid maltase deficiency, 4 with sporadic, adult-onset idiopathic "autophagic" vacuolar myopathy (that is not acid-maltase deficient) and one with abnormal mitochondria, but in only one of greater than 50 other cultures of normal and denervated human muscle. They were also induced abundantly in cultured normal human muscle by exposure to 0.5 mM DNP.
在8例酸性麦芽糖酶缺乏患者、4例散发性成人起病的特发性“自噬性”空泡性肌病(非酸性麦芽糖酶缺乏)患者以及1例线粒体异常患者的培养肌纤维中,经常观察到薄肌节,其为由非常细的细丝束组成的分层结构,这些细丝束被宽20 - 80nm的周期性横向致密线分隔成约260nm宽的带,但在超过50份正常和去神经支配的人类肌肉的其他培养物中,仅在其中1份中观察到。通过暴露于0.5mM二硝基酚(DNP),它们在培养的正常人类肌肉中也大量诱导产生。