SPTLC1 is mutated in hereditary sensory neuropathy, type 1

Hereditary sensory neuropathy type 1 (HSN1, MIM 162400; ref. 1) genetically maps to human chromosome 9q22 (refs. 2-4). We report here that the gene encoding a subunit of serine palmitoyltransferase is located within the HSN1 locus, expressed in dorsal root ganglia (DRG) and mutated in HSN1.     

Mox2 is a component of the genetic hierarchy controlling limb muscle development.

The skeletal muscles of the limbs develop from myogenic progenitors that originate in the paraxial mesoderm and migrate into the limb-bud mesenchyme. Among the genes known to be important for muscle development in mammalian embryos are those encoding the basic helix-loop-helix (bHLH) myogenic regulatory factors (MRFs; MyoD, Myf5, myogenin and MRF4) and Pax3, a paired-type homeobox gene that is critical for the development of limb musculature. Mox1 and Mox2 are closely related homeobox genes that are expressed in overlapping patterns in the paraxial mesoderm and its derivatives. Here we show that mice homozygous for a null mutation of Mox2 have a developmental defect of the limb musculature, characterized by an overall reduction in muscle mass and elimination of specific muscles. Mox2 is not needed for the migration of myogenic precursors into the limb bud, but it is essential for normal appendicular muscle formation and for the normal regulation of myogenic genes, as demonstrated by the downregulation of Pax3 and Myf5 but not MyoD in Mox2-deficient limb buds. Our findings show that the MOX2 homeoprotein is an important regulator of vertebrate limb myogenesis.     

禁带区域内负的群速度

研究超声波经过两个烧结样品的行为.两个烧结样品为有序的面心立方结构,它们的结构几乎相同.研究表明:在两个有序的准均匀的介质中分别有一个禁带区域.就禁带区域而言,在两个样品中产生禁带的区域非常接近.同时在禁带出现的区域群速度为负值.我们的结果表明群速度的负值的确存在于有序介质中的禁带区域内.     

Discovery of water vapour around IRC+10216 as evidence for comets orbiting another star.

Since 1995, planets with masses comparable to that of Jupiter have been discovered around approximately 60 stars. These planets have not been seen directly, but their presence has been inferred from the small reflex motions that they gravitationally induce on the star they orbit; these motions result in small periodic wavelength shifts in the stellar spectrum. The presence of analogues of the smaller bodies in our Solar System cannot, however, be determined using this technique, because the induced reflex motions are too small-so an alternative approach is needed. Here we report the observation of circumstellar water vapour around the ageing carbon star IRC+10216; water is not expected in measurable quantities around such a star. The only plausible explanation for this water is that the recent evolution of IRC+10216, which has been accompanied by a prodigious increase in its luminosity, is causing the vaporization of a collection of orbiting icy bodies-a process considered in an earlier theoretical study.     

Relativistic Effects in the Global Positioning System

l'INTRo~TloNThepurposeofthisworkistoexplainhowrelativisticeffectsareaccountedforintheGlobalPositioningSystem(GPS).AlthoughclockvelocitiesaresmallandgravitationalfieldsareweakneartheEarth,theygiverisetosignificantrelativisticeffects.Theseeffectsincludesecond--orderDopplerfrequencyshiftsofclocksduetotheirrelativevelocities,gravitationalfrequencyshifts,andtheSagnaceffectduetotheEarth'srotation.Ifsucheffectsarenotaccountedforproperly,theGPSsimplywouldnotworkatallwell.Thesuccessandself--con…     

A massive, cooling-flow-induced starburst in the core of a luminous cluster of galaxies

In the cores of some clusters of galaxies the hot intracluster plasma is dense enough that it should cool radiatively in the cluster's lifetime, leading to continuous 'cooling flows' of gas sinking towards the cluster centre, yet no such cooling flow has been observed. The low observed star-formation rates and cool gas masses for these 'cool-core' clusters suggest that much of the cooling must be offset by feedback to prevent the formation of a runaway cooling flow. Here we report X-ray, optical and infrared observations of the galaxy cluster SPT-CLJ2344-4243 (ref. 11) at redshift z = 0.596. These observations reveal an exceptionally luminous (8.2?×?10(45)?erg?s(-1)) galaxy cluster that hosts an extremely strong cooling flow (around 3,820 solar masses a year). Further, the central galaxy in this cluster appears to be experiencing a massive starburst (formation of around 740 solar masses a year), which suggests that the feedback source responsible for preventing runaway cooling in nearby cool-core clusters may not yet be fully established in SPT-CLJ2344-4243. This large star-formation rate implies that a significant fraction of the stars in the central galaxy of this cluster may form through accretion of the intracluster medium, rather than (as is currently thought) assembling entirely via mergers.     

An almost head-on collision as the origin of two off-centre rings in the Andromeda galaxy

The unusual morphology of the Andromeda galaxy (Messier 31, the closest spiral galaxy to the Milky Way) has long been an enigma. Although regarded for decades as showing little evidence of a violent history, M31 has a well-known outer ring of star formation at a radius of ten kiloparsecs whose centre is offset from the galaxy nucleus. In addition, the outer galaxy disk is warped, as seen at both optical and radio wavelengths. The halo contains numerous loops and ripples. Here we report the presence of a second, inner dust ring with projected dimensions of 1.5 x 1 kiloparsecs and offset by about half a kiloparsec from the centre of the galaxy (based upon an analysis of previously-obtained data). The two rings appear to be density waves propagating in the disk. Numerical simulations indicate that both rings result from a companion galaxy plunging through the centre of the disk of M31. The most likely interloper is M32. Head-on collisions between galaxies are rare, but it appears nonetheless that one took place 210 million years ago in our Local Group of galaxies.