Conformational differences in protein disulfide linkages between normal hair and hair from subjects with trichothiodystrophy: A quantitative analysis by Raman microspectroscopy

Abstract

Raman spectra of normal hair shafts and hair shafts from patients exhibiting trichothiodystrophy (TTD) were obtained using line focus laser illumination. Because hair from TTD patients has a significant decrease in the content of the sulfur-containing amino acids in comparison to normal hair, the 550-500 cm(-1) disulfide stretching mode region of the Raman spectrum was examined in detail. A quantitative spectral analysis demonstrates significant increases in the two energetically less favored gauche-gauche-trans (g-g-t) and trans-gauche-trans (t-g-t) forms. These observations suggest that the increased amounts of these less stable disulfide conformers are contributing factors to or associated with the hair brittleness observed for this congenital disorder. Structure-spectra correlations for the three dominant disulfide conformers are confirmed by quantum chemical calculations using modern density functional theory (DFT).

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

“…96,205 Polarization microscopy can demonstrate the tiger-tail pattern of TTD, 217,218 which corresponds to the diagnostic low-sulfur protein content of the hair. 219,220 Special immunohistochemical procedures can be combined, eg, to confirm filaggrin deficiency in IV, 202,221 or demonstrate absent or reduced expression of LEKTI that supports the diagnosis of NS. [222][223][224] To screen for TGase-1 deficiency in ARCI unfixed cryostat sections are used for the enzyme activity assay.…”

Measuring changes in chemistry, composition, and molecular structure within hair fibers by infrared and Raman spectroscopic imaging

“…[7][8][9][10][11] The inherent distinct sensitivities of these two techniques to molecular vibrations provide complementary structure and chemistry information. IR spectroscopic imaging studies of hair cross sections have been reported with synchrotron 12 and micro attenuated total reflection infrared microscopy.…”

Analysis of internal structure changes in black human hair keratin fibers with aging using Raman spectroscopy

“…It has been found that the SÀ ÀS intensity of the hair from subjects with trichothiodystrophy (Brittle hair that has a cystine content of less than 50% compared with that of normal hair 33 ) significantly decreased compared with that of normal hair. 28 Considering this, it would be interesting and worthwhile to further investigate the internal structure changes of black human hair from Japanese females in their seventies or eighties due to aging. …”

“…The direct characterization of keratin fibers has been performed using X-ray diffraction, 1,[7][8][9][10][11][12] solid state NMR, 8,13 Raman, [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] and Infrared spectroscopy. 14,16,17 Information about the amorphous region (the cuticle and matrix) of…”

“…[20][21][22][23][24][25][26] Most importantly, by using this method, information about the crystalline fibrous (microfibril) and amorphous (matrix) structures existing in the cortex region can be obtained. However, up to now, Raman spectroscopic analysis had been limited to white human hair, [20][21][22][23][24][25][26] which has no melanin granules, and blond human hair, 27,28 which has few melanin granules. The characterization of virgin black human hair that has high melanin granule content had been impossible, because of sample destruction due to laser exposure and an increasing baseline resulting from fluorescence.…”