Stabilizing mutations of KLHL24 ubiquitin ligase cause loss of keratin 14 and human skin fragility

2016 ◽  
Vol 48 (12) ◽  
pp. 1508-1516 ◽  
Author(s):  
Zhimiao Lin ◽  
Shuo Li ◽  
Cheng Feng ◽  
Shang Yang ◽  
Huijun Wang ◽  
...  
Keyword(s):  
Human Skin ◽  
Ubiquitin Ligase ◽  
Keratin 14 ◽  
Skin Fragility

scholarly journals 474 Stabilizing mutations of KLHL24 ubiquitin ligase cause loss of keratin 14 and human skin fragility

2017 ◽  
Vol 137 (5) ◽  
pp. S82
Author(s):  
C. Feng ◽  
Z. Lin ◽  
S. Li ◽  
H. Wang ◽  
Y. Yang ◽  
...  
Keyword(s):  
Human Skin ◽  
Ubiquitin Ligase ◽  
Keratin 14 ◽  
Skin Fragility

scholarly journals Revertant mosaicism in a human skin fragility disorder results from slipped mispairing and mitotic recombination

2012 ◽  
Vol 122 (5) ◽  
pp. 1742-1746 ◽  
Author(s):  
Dimitra Kiritsi ◽  
Yinghong He ◽  
Anna M.G. Pasmooij ◽  
Meltem Onder ◽  
Rudolf Happle ◽  
...  
Keyword(s):  
Human Skin ◽  
Mitotic Recombination ◽  
Skin Fragility

A web-like network of type vi collagen in human skin is revealed by immuno-electron microscopy

1988 ◽  
Vol 46 ◽  
pp. 382-383
Author(s):  
Douglas R. Keene ◽  
Robert W. Glanville ◽  
Eva Engvall
Keyword(s):  
Electron Microscopy ◽  
Monoclonal Antibody ◽  
Human Skin ◽  
Basement Membranes ◽  
Primary Antibody ◽  
Fat Cells ◽  
Secondary Antibody ◽  
Type Vi Collagen ◽  
Dermal Matrix ◽  
Immuno Electron Microscopy

A mouse monoclonal antibody (5C6) prepared against human type VI collagen (1) has been used in this study to immunolocalize type VI collagen in human skin. The enbloc method used involves exposing whole tissue pieces to primary antibody and 5 nm gold conjugated secondary antibody before fixation, and has been described in detail elsewhere (2).Biopsies were taken from individuals ranging in age from neonate to 65 years old. By immuno-electron microscopy, type VI collagen is found to be distributed as a fine branching network closely associated with (but not attached to) banded collagen fibrils containing types I and III collagen (Fig. 1). It appears to enwrap fibers, to weave between individual fibrils within a fiber, and to span the distance separating fibers, creating a “web-like network” which entraps fibers within deep papillary and reticular dermal layers (Fig. 2). Relative to that in the dermal matrix, the concentration of type VI collagen is higher around endothelial basement membranes limiting the outer boundaries of nerves, capillaries, and fat cells (Fig. 3).

Effect of Acetone and Kerosene on Skin Ultrastructure

1972 ◽  
Vol 30 ◽  
pp. 92-93
Author(s):  
A. P. Lupulescu ◽  
H. Pinkus ◽  
D. J. Birmingham
Keyword(s):  
Electron Microscopy ◽  
Human Skin ◽  
Osmium Tetroxide ◽  
Human Epidermis ◽  
Ultrastructural Changes ◽  
Chemical Agents ◽  
Skin Ultrastructure ◽  
Volar Surface

Our laboratory is engaged in the study of the effect of different chemical agents on human skin, using electron microscopy. Previous investigations revealed that topical use of a strong alkali (NaOH 1N) or acid (HCl 1N), induces ultrastructural changes in the upper layers of human epidermis. In the current experiments, acetone and kerosene, which are primarily lipid solvents, were topically used on the volar surface of the forearm of Caucasian and Negro volunteers. Skin specimens were bioptically removed after 90 min. exposure and 72. hours later, fixed in 3% buffered glutaraldehyde, postfixed in 1% phosphate osmium tetroxide, then flat embedded in Epon.

Changes in corneocyte element concentrations occur in skin inner stratum corneum

1990 ◽  
Vol 48 (2) ◽  
pp. 146-147
Author(s):  
R. R. Warner
Keyword(s):  
Stratum Corneum ◽  
Human Skin ◽  
Element Concentration ◽  
Skin Barrier ◽  
Barrier Properties ◽  
Brick Wall ◽  
Inorganic Elements ◽  
Dry Skin ◽  
Skin Biopsies ◽  
Intercellular Lipid

Keratinocytes undergo maturation during their transit through the viable layers of skin, and then abruptly transform into flattened, anuclear corneocytes that constitute the cellular component of the skin barrier, the stratum corneum (SC). The SC is generally considered to be homogeneous in its structure and barrier properties, and is often shown schematically as a featureless brick wall, the “bricks” being the corneocytes, the “mortar” being intercellular lipid. Previously we showed the outer SC was not homogeneous in its composition, but contained steep gradients of the physiological inorganic elements Na, K and Cl, likely originating from sweat salts. Here we show the innermost corneocytes in human skin are also heterogeneous in composition, undergoing systematic changes in intracellular element concentration during transit into the interior of the SC.Human skin biopsies were taken from the lower leg of individuals with both “good” and “dry” skin and plunge-frozen in a stirred, cooled isopentane/propane mixture.

Desmosomal distribution in the epidermis of a non-contracting human skin equivalent

1992 ◽  
Vol 50 (1) ◽  
pp. 896-897
Author(s):  
L.X. Oakford ◽  
S.D. Dimitrijevich ◽  
R. Gracy
Keyword(s):  
Human Skin ◽  
Basal Layer ◽  
Skin Equivalent ◽  
Tissue Component ◽  
Intact Skin ◽  
Similar Sequence ◽  
Sequence Of Events ◽  
Suprabasal Keratinocytes ◽  
Human Skin Equivalent

In intact skin the epidermal layer is a dynamic tissue component which is maintained by a basal layer of mitotically active cells. The protective upper epidermis, the stratum corneum, is generated by differentiation of the suprabasal keratinocytes which eventually desquamate as anuclear comeocytes. A similar sequence of events is observed in vitro in the non-contracting human skin equivalent (HSE) which was developed in this lab (1). As a part of the definition process for this model of living skin we are examining its ultrastructural features. Since desmosomes are important in maintaining cell-cell interactions in stratified epithelia their distribution in HSE was examined.

Novel players fine-tune plant trade-offs

2015 ◽  
Vol 58 ◽  
pp. 83-100 ◽  
Author(s):  
Selena Gimenez-Ibanez ◽  
Marta Boter ◽  
Roberto Solano
Keyword(s):  
Ubiquitin Ligase ◽  
Feedback Loop ◽  
Molecular Level ◽  
26S Proteasome ◽  
Signalling Molecules ◽  
Transcriptional Reprogramming ◽  
Trade Offs ◽  
Jaz Proteins ◽  
Regulatory Feedback Loop ◽  
Fine Tune

Jasmonates (JAs) are essential signalling molecules that co-ordinate the plant response to biotic and abiotic challenges, as well as co-ordinating several developmental processes. Huge progress has been made over the last decade in understanding the components and mechanisms that govern JA perception and signalling. The bioactive form of the hormone, (+)-7-iso-jasmonyl-l-isoleucine (JA-Ile), is perceived by the COI1–JAZ co-receptor complex. JASMONATE ZIM DOMAIN (JAZ) proteins also act as direct repressors of transcriptional activators such as MYC2. In the emerging picture of JA-Ile perception and signalling, COI1 operates as an E3 ubiquitin ligase that upon binding of JA-Ile targets JAZ repressors for degradation by the 26S proteasome, thereby derepressing transcription factors such as MYC2, which in turn activate JA-Ile-dependent transcriptional reprogramming. It is noteworthy that MYCs and different spliced variants of the JAZ proteins are involved in a negative regulatory feedback loop, which suggests a model that rapidly turns the transcriptional JA-Ile responses on and off and thereby avoids a detrimental overactivation of the pathway. This chapter highlights the most recent advances in our understanding of JA-Ile signalling, focusing on the latest repertoire of new targets of JAZ proteins to control different sets of JA-Ile-mediated responses, novel mechanisms of negative regulation of JA-Ile signalling, and hormonal cross-talk at the molecular level that ultimately determines plant adaptability and survival.

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