DYT11 myoclonus-dystonia (M-D) is a movement disorder characterized by myoclonic jerks with dystonic symptoms and caused by mutations in paternally expressed SGCE, which codes for ε-sarcoglycan. Paternally inherited Sgce heterozygous knock-out (KO) mice exhibit motor deficits and spontaneous myoclonus. Abnormal nuclear envelopes have been reported in cellular and mouse models of early-onset DYT...

Abstract For a split semisimple algebraic group H with its maximal torus S , let be the restriction homomorphism of Chow rings classifying spaces and where W is Weyl group. A constraint on image f given by Steenrod operations, has been applied to spin groups in previous paper. Here, we describe apply another constraint, which reductive envelopes . We also recover this way some older results ort...

We present a framework for the systematic study of parametric variation in planar mechanical parts and for efficiently computing approximations of their tolerance envelopes. Part features are specified by explicit functions defining their position and shape as a function of parameters whose nominal values vary along tolerance intervals. Their tolerance envelopes model perfect form least and mos...

In this paper, we present a speech and audio analysis-synthesis method based on a Basilar Membrane (BM) model. The audio signal is represented in this method by the Hilbert envelopes of the responses to complex gammatone filters uniformally spaced on a critical band scale. We show that for speech and audio signals, a perceptually equivalent signal can be reconstructed from the envelopes alone b...

A study of the chemical structure of the envelopes around a sample protostars is introduced. Physical models for the envelopes derived using 1D radiative transfer modeling of their dust continuum emission are used as input for Monte Carlo modeling of single-dish line observations in order to establish the chemical inventories for the different sources. CO and HCO abundances are found to be corr...

We present a feature extraction technique based on static and dynamic modulation spectrum derived from long-term envelopes in sub-bands. Estimation of the sub-band temporal envelopes is done using Frequency Domain Linear Prediction (FDLP). These sub-band envelopes are compressed with a static (logarithmic) and dynamic (adaptive loops) compression. The compressed sub-band envelopes are transform...

Epidermal keratinocytes, late in their terminal differentiation, form cross-linked envelopes resistant to ionic detergent and reducing agent. Because the cross-linking process is catalyzed by the keratinocyte transglutaminase, the absence of active transglutaminase should result in failure of the keratinocyte to form a cross-linked envelope. Three keratinocyte strains bearing mutations in the k...