vacuoles

Vesicles, vacuoles and plant cell walls

Vesicles: membrane-bound sacs found in cells (can be visualised as a bubble of liquid). They are made up of a variety of molecules and can form naturally or prepared. They are used to store or carry different substances around the cell. Most vesicles have specialised functions, depending on the materials they contain.

Vacuoles: found in all plant and fungal cells and maintains cell stability. It is filled with water containing organic and inorganic molecules so that it pushes the cytoplasm against the cell wall, making the cell turgid and helps support the plant.

Plant cell walls: found on the outside of plant cell surface membranes. It is made up of cellulose, which forms a sieve-like network of strands. This makes the cell wall strong. It is held rigid by the pressure of the fluid inside the cell (turgor pressure) so it supports the cell and the whole plant. 

Definitions:

  • Turgid-describes a cell that is full of water and the pressure of the cell wall prevents more water entering.

anonymous asked:

could you imagine the internal anatomy of your time lord dragons as being dimensionally transcendental?, given that time lord technology is about being bigger than the inside, it may need sketches of it.

i’ve been trying to think of a response for this. um. i just don’t get it. why would your internal anatomy need to be dimensionally transcendental? like, okay, there have been instances at the mexicatessen where that carne asada could have done with additional breathing space but i’m at a loss as to what’s going on here. lets fill all these extra dimensionally transcendental vacuoles w/air and make u super light? stuff u full of lungs so u can breathe forever? ruminate? store spare change?

if u do sketches feel free to submit them, i’m quite interested in what you have in mind

GNE myopathy associated with congenital thrombocytopenia: a report of two siblings.

PubMed:
Related Articles

GNE myopathy associated with congenital thrombocytopenia: a report of two siblings.

Neuromuscul Disord. 2014 Dec;24(12):1068-72

Authors: Izumi R, Niihori T, Suzuki N, Sasahara Y, Rikiishi T, Nishiyama A, Nishiyama S, Endo K, Kato M, Warita H, Konno H, Takahashi T, Tateyama M, Nagashima T, Funayama R, Nakayama K, Kure S, Matsubara Y, Aoki Y, Aoki M

Abstract
GNE myopathy is an autosomal recessive muscular disorder caused by mutations in the gene encoding the key enzyme in sialic acid biosynthesis, UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/MNK). Here, we report two siblings with myopathy with rimmed vacuoles and congenital thrombocytopenia who harbored two compound heterozygous GNE mutations, p.V603L and p.G739S. Thrombocytopenia, which is characterized by shortened platelet lifetime rather than ineffective thrombopoiesis, has been observed since infancy. We performed exome sequencing and array CGH to identify the underlying genetic etiology of thrombocytopenia. No pathogenic variants were detected among the known causative genes of recessively inherited thrombocytopenia; yet, candidate variants in two genes that followed an autosomal recessive mode of inheritance, including previously identified GNE mutations, were detected. Alternatively, it is possible that the decreased activity of GNE/MNK itself, which would lead to decreased sialic content in platelets, is associated with thrombocytopenia in these patients. Further investigations are required to clarify the association between GNE myopathy and the pathogenesis of thrombocytopenia.

PMID: 25257349 [PubMed - indexed for MEDLINE] http://dlvr.it/BgjvqH

biOasis Announces the Successful Delivery to the CNS of an Enzyme Used to Treat Hunter Syndrome

VANCOUVER, BRITISH COLUMBIA–(Marketwired - Jul 31, 2015) - BIOASIS TECHNOLOGIES INC. (BIOAF)(TSX VENTURE:BTI), a pioneering biopharmaceutical company focused on overcoming the limitations of therapeutic drug delivery across the blood-brain barrier (BBB), announces the completion and assessment of the data in the knockout mouse model of Hunter Syndrome (MPS II) performed in Padova, Italy, by a team led by Dr. Maurizio Scarpa of the Brains for Brain Foundation.

This knockout mouse model of Hunter Syndrome (MPS II) showed that the Transcend fusion protein, MTfp-I2S (MTfp-Iduronate-2-sulfatase), was able to cross the blood-brain barrier, reached therapeutic levels in the CNS and significantly decreased the number of lysosomal storage vacuoles in the cytoplasm of brain cells.

The study also showed that MTfp-I2S normalizes the GAG level in the periphery (liver & urine) showing that the recombinant enzyme is active and distributed throughout the body. The MTfp-I2S treatments were safe, showing no toxicity, and the activity of the enzyme was not affected by the MTfp peptide carrier.

Sufferers of the Lysosomal Storage Disease, Mucopolysaccharidoses Type II (MPS II or Hunter Syndrome), are unable to produce sufficient quantities of the enzyme Iduronate-2-sulfatase (I2S), resulting in the impaired degradation and removal of glycosaminoglycans (GAGs) from the body. The resulting accumulation of lysosomal storage vacuoles in cells leads to the death of these cells, causing severe physical and mental impairments.

Dr. Maurizio Scarpa, President of the Brains for Brain Foundation stated, “The study that we have recently performed in collaboration with biOasis produced some exciting data. I am very encouraged that after some adjustments regarding dosing, we might be able to move this program into the clinic. My goal and I am sure the goal of biOasis as well, is to help children with this devastating disease. I am very encouraged that based on these results, Transcend may play an important role in helping us achieve our goals.”

CEO Rob Hutchison commented, “It has been a pleasure working with Dr. Scarpa and the Brains for Brain foundation. When we looked to advance our Lysosomal Storage Disease program, Dr. Scarpa along with Dr. Begley where our first choices and we were very pleased when, through their foundation, Brains for Brain, they agreed to work with us. Working with these world leaders and their team at the University of Padova has been very exciting for us. The data that they have generated has provided biOasis with confirmation that we’ve been on the right track with our Transcend peptide carrier and we are now poised to potentially help make a difference to children coping with this awful disease.”

About The Brains for Brain Foundation

Mission: B4B aims to develop new and innovative therapeutic strategies to cross the Blood-Brain Barrier, a capillary system that shields and defends the CNS from circulating neurotoxins. It has a very important filtering protective function but unfortunately also prevents access to the brain by most candidate therapeutic drugs under development for CNS diseases. The B4B mission consists of the promotion of an International Network of health professionals in rare disorders, specifically in the field of rare neurological pediatric diseases. The purposes of the FOUNDATION are to support the following activities in the field of rare Neurological Disorders: - scientific research - knowledge dissemination - social and socio-medical assistance - health assistance.

About biOasis

biOasis Technologies Inc. is a biopharmaceutical company headquartered in Richmond, Canada focused on overcoming the limitations of therapeutic drug delivery across the BBB. The company is developing and commercializing a proprietary brain delivery technology to address unmet medical needs in the treatment of central nervous system disorders. biOasis trades on the OTCQX under the symbol “BIOAF” and on the TSX Venture Exchange under the symbol “BTI”. For more information about the company please visit www.bioasis.ca.

Forward-Looking Statements

Certain statements in this press release contain forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995 or forward-looking information under applicable Canadian securities legislation that may not be based on historical fact, including without limitation statements containing the words “believe”, “may”, “plan”, “will”, “estimate”, “continue”, “anticipate”, “intend”, “expect” and similar expressions. Such forward-looking statements or information involve known and unknown risks, uncertainties and other factors that may cause our actual results, events or developments, or industry results, to be materially different from any future results, events or developments express or implied by such forward-looking statements or information. Such factors include, among others, our stage of development, lack of any product revenues, additional capital requirements, risk associated with the completion of clinical trials and obtaining regulatory approval to market our products, the ability to protect our intellectual property, dependence on collaborative partners and the prospects for negotiating additional corporate collaborations or licensing arrangements and their timing. Specifically, certain risks and uncertainties that could cause such actual events or results expressed or implied by such forward-looking statements and information to differ materially from any future events or results expressed or implied by such statements and information include, but are not limited to, the risks and uncertainties that: products that we develop may not succeed in preclinical or clinical trials, or future products in our targeted corporate objectives; our future operating results are uncertain and likely to fluctuate; we may not be able to raise additional capital; we may not be successful in establishing additional corporate collaborations or licensing arrangements; we may not be able to establish marketing and the costs of launching our products may be greater than anticipated; we have no experience in commercial manufacturing; we may face unknown risks related to intellectual property matters; we face increased competition from pharmaceutical and biotechnology companies; and other factors as described in detail in our filings with the Canadian securities regulatory authorities at www.sedar.com. Given these risks and uncertainties, you are cautioned not to place undue reliance on such forward-looking statements and information, which are qualified in their entirety by this cautionary statement. All forward-looking statements and information made herein are based on our current expectations and we undertake no obligation to revise or update such forward- looking statements and information to reflect subsequent events or circumstances, except as required by law.

On Behalf of the Board of Directors

Rob Hutchison, Chairman & CEO

“Neither the TSX Venture Exchange nor its Regulation Services Provider (as that term is defined in the policies of the TSX Venture Exchange) accepts responsibility for the adequacy or accuracy of this release.”

biOasis Announces the Successful Delivery to the CNS of an Enzyme Used to Treat Hunter Syndrome

VANCOUVER, BRITISH COLUMBIA–(Marketwired - July 31, 2015) - BIOASIS TECHNOLOGIES INC. (BIOAF)(TSX VENTURE:BTI), a pioneering biopharmaceutical company focused on overcoming the limitations of therapeutic drug delivery across the blood-brain barrier (BBB), announces the completion and assessment of the data in the knockout mouse model of Hunter Syndrome (MPS II) performed in Padova, Italy, by a team led by Dr. Maurizio Scarpa of the Brains for Brain Foundation. 

This knockout mouse model of Hunter Syndrome (MPS II) showed that the Transcend fusion protein, MTfp-I2S (MTfp-Iduronate-2-sulfatase), was able to cross the blood-brain barrier, reached therapeutic levels in the CNS and significantly decreased the number of lysosomal storage vacuoles in the cytoplasm of brain cells.

The study also showed that MTfp-I2S normalizes the GAG level in the periphery (liver & urine) showing that the recombinant enzyme is active and distributed throughout the body. The MTfp-I2S treatments were safe, showing no toxicity, and the activity of the enzyme was not affected by the MTfp peptide carrier.

Sufferers of the Lysosomal Storage Disease, Mucopolysaccharidoses Type II (MPS II or Hunter Syndrome), are unable to produce sufficient quantities of the enzyme Iduronate-2-sulfatase (I2S), resulting in the impaired degradation and removal of glycosaminoglycans (GAGs) from the body. The resulting accumulation of lysosomal storage vacuoles in cells leads to the death of these cells, causing severe physical and mental impairments.

Dr. Maurizio Scarpa, President of the Brains for Brain Foundation stated, “The study that we have recently performed in collaboration with biOasis produced some exciting data. I am very encouraged that after some adjustments regarding dosing, we might be able to move this program into the clinic. My goal and I am sure the goal of biOasis as well, is to help children with this devastating disease. I am very encouraged that based on these results, Transcend may play an important role in helping us achieve our goals.”

CEO Rob Hutchison commented, “It has been a pleasure working with Dr. Scarpa and the Brains for Brain foundation. When we looked to advance our Lysosomal Storage Disease program, Dr. Scarpa along with Dr. Begley where our first choices and we were very pleased when, through their foundation, Brains for Brain, they agreed to work with us. Working with these world leaders and their team at the University of Padova has been very exciting for us. The data that they have generated has provided biOasis with confirmation that we’ve been on the right track with our Transcend peptide carrier and we are now poised to potentially help make a difference to children coping with this awful disease.”

About The Brains for Brain Foundation

Mission: B4B aims to develop new and innovative therapeutic strategies to cross the Blood-Brain Barrier, a capillary system that shields and defends the CNS from circulating neurotoxins. It has a very important filtering protective function but unfortunately also prevents access to the brain by most candidate therapeutic drugs under development for CNS diseases. The B4B mission consists of the promotion of an International Network of health professionals in rare disorders, specifically in the field of rare neurological pediatric diseases. The purposes of the FOUNDATION are to support the following activities in the field of rare Neurological Disorders: - scientific research - knowledge dissemination - social and socio-medical assistance - health assistance.

About biOasis

biOasis Technologies Inc. is a biopharmaceutical company headquartered in Richmond, Canada focused on overcoming the limitations of therapeutic drug delivery across the BBB. The company is developing and commercializing a proprietary brain delivery technology to address unmet medical needs in the treatment of central nervous system disorders. biOasis trades on the OTCQX under the symbol “BIOAF” and on the TSX Venture Exchange under the symbol “BTI”. For more information about the company please visit www.bioasis.ca.

Forward-Looking Statements

Certain statements in this press release contain forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995 or forward-looking information under applicable Canadian securities legislation that may not be based on historical fact, including without limitation statements containing the words “believe”, “may”, “plan”, “will”, “estimate”, “continue”, “anticipate”, “intend”, “expect” and similar expressions. Such forward-looking statements or information involve known and unknown risks, uncertainties and other factors that may cause our actual results, events or developments, or industry results, to be materially different from any future results, events or developments express or implied by such forward-looking statements or information. Such factors include, among others, our stage of development, lack of any product revenues, additional capital requirements, risk associated with the completion of clinical trials and obtaining regulatory approval to market our products, the ability to protect our intellectual property, dependence on collaborative partners and the prospects for negotiating additional corporate collaborations or licensing arrangements and their timing. Specifically, certain risks and uncertainties that could cause such actual events or results expressed or implied by such forward-looking statements and information to differ materially from any future events or results expressed or implied by such statements and information include, but are not limited to, the risks and uncertainties that: products that we develop may not succeed in preclinical or clinical trials, or future products in our targeted corporate objectives; our future operating results are uncertain and likely to fluctuate; we may not be able to raise additional capital; we may not be successful in establishing additional corporate collaborations or licensing arrangements; we may not be able to establish marketing and the costs of launching our products may be greater than anticipated; we have no experience in commercial manufacturing; we may face unknown risks related to intellectual property matters; we face increased competition from pharmaceutical and biotechnology companies; and other factors as described in detail in our filings with the Canadian securities regulatory authorities at www.sedar.com. Given these risks and uncertainties, you are cautioned not to place undue reliance on such forward-looking statements and information, which are qualified in their entirety by this cautionary statement. All forward-looking statements and information made herein are based on our current expectations and we undertake no obligation to revise or update such forward- looking statements and information to reflect subsequent events or circumstances, except as required by law.

On Behalf of the Board of Directors

Rob Hutchison, Chairman & CEO

“Neither the TSX Venture Exchange nor its Regulation Services Provider (as that term is defined in the policies of the TSX Venture Exchange) accepts responsibility for the adequacy or accuracy of this release.”

SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.

PubMed: Related Articles

SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.

Neurology. 2015 Jul 24;

Authors: Bucelli RC, Arhzaouy K, Pestronk A, Pittman SK, Rojas L, Sue CM, Evilä A, Hackman P, Udd B, Harms MB, Weihl CC

Abstract
OBJECTIVE: To identify the genetic etiology and characterize the clinicopathologic features of a novel distal myopathy.
METHODS: We performed whole-exome sequencing on a family with an autosomal dominant distal myopathy and targeted exome sequencing in 1 patient with sporadic distal myopathy, both with rimmed vacuolar pathology. We also evaluated the pathogenicity of identified mutations using immunohistochemistry, Western blot analysis, and expression studies.
RESULTS: Sequencing identified a likely pathogenic c.1165+1 G>A splice donor variant in SQSTM1 in the affected members of 1 family and in an unrelated patient with sporadic distal myopathy. Affected patients had late-onset distal lower extremity weakness, myopathic features on EMG, and muscle pathology demonstrating rimmed vacuoles with both TAR DNA-binding protein 43 and SQSTM1 inclusions. The c.1165+1 G>A SQSTM1 variant results in the expression of 2 alternatively spliced SQSTM1 proteins: 1 lacking the C-terminal PEST2 domain and another lacking the C-terminal ubiquitin-associated (UBA) domain, both of which have distinct patterns of cellular and skeletal muscle localization.
CONCLUSIONS: SQSTM1 is an autophagic adaptor that shuttles aggregated and ubiquitinated proteins to the autophagosome for degradation via its C-terminal UBA domain. Similar to mutations in VCP, dominantly inherited mutations in SQSTM1 are now associated with rimmed vacuolar myopathy, Paget disease of bone, amyotrophic lateral sclerosis, and frontotemporal dementia. Our data further suggest a pathogenic connection between the disparate phenotypes.

PMID: 26208961 [PubMed - as supplied by publisher] http://dlvr.it/Bf99nL

Leigh syndrome: neuropathology and pathogenesis.

PubMed:
Related Articles

Leigh syndrome: neuropathology and pathogenesis.

J Neuropathol Exp Neurol. 2015 Jun;74(6):482-92

Authors: Lake NJ, Bird MJ, Isohanni P, Paetau A

Abstract
Leigh syndrome (LS) is the most common pediatric presentation of a defined mitochondrial disease. This progressive encephalopathy is characterized pathologically by the development of bilateral symmetrical lesions in the brainstem and basal ganglia that show gliosis, vacuolation, capillary proliferation, relative neuronal preservation, and by hyperlacticacidemia in the blood and/or cerebrospinal fluid. Understanding the molecular mechanisms underlying this unique pathology has been challenging, particularly in view of the heterogeneous and not yet fully determined genetic basis of LS. Moreover, animal models that mimic features of LS have only been created relatively recently. Here, we review the pathology of LS and consider what might be the molecular mechanisms underlying its pathogenesis. Data from a wide range of sources, including patient samples, animal models, and studies of hypoxic-ischemic encephalopathy (a condition that shares features with LS), were used to provide insight into the pathogenic mechanisms that may drive lesion development. Based on current data, we suggest that severe ATP depletion, gliosis, hyperlacticacidemia, reactive oxygen species, and potentially excitotoxicity cumulatively contribute to the neuropathogenesis of LS. An intimate understanding of the molecular mechanisms causing LS is required to accelerate the development of LS treatments.

PMID: 25978847 [PubMed - indexed for MEDLINE] http://dlvr.it/BgTdHg

Fenugreek seeds can be used as a regular nutrient to alleviate the side effects of ethanol ingestion, in the kidneys, especially for chronic renal failure patients.

PMID:  Rom J Morphol Embryol. 2015 ;56(2):445-51. PMID: 26193212 Abstract Title:  Fenugreek powder exerts protective effects on alcoholised rats’ kidney, highlighted using ultrastructural studies. Abstract:  Trigonella foenum-graecum (TFG) seeds exert a protective antioxidant effect and membrane protector through their rich content in polyphenolic flavonoids. The previous research focused on the hypoglycemic action of the seeds, with scarce studies on the preventive effects in the pathology of the kidney. Our work was conducted on an experimental in vivo model; the animals were given two different concentrations of TFG seeds, consequently to alcohol intoxication. Transmission electron microscopy (TEM) analysis showed vacuolation in cytoplasm, edemas at the apical pole of the nephrocytes, diffusion of the cytoplasmic and mitochondrial matrix and the increase in number of the lysozymes and especially peroxisomes, as well as the congestion of blood capillaries. In the case of the groups T5R and T10R, which received Trigonella powder together with ethanol, the structural and ultrastructural changes produced by the ethylic intoxication were more reduced, being somewhat improved in the T5R group. Therefore, the majority of the cells nuclei have retained their spherical shape, being at the same time predominantly euchromatic, with little heterochromatin and evenly dispersed. Our results suggest the use of Trigonella seeds as a food supplement to prevent cellular deterioration and improve renal morphology and function. http://j.mp/1OoWyq7

Because incorrectmarauderquotes​ say everybody was tagged, so i guess i’m too.

Nickname: Consty, but Majestic Douchebag is actually my favourite nickname, ‘cause it’ pretty accurate. I’m a douchebag.
Zodiac sign: Scorpio.
Gender: Agender
Sexual orientation: Homoflexible demisexual..?
Favourite colour: According to my closet, it’s black.
Current time: 07:07AM
Average amount of sleep: Oh man, i don’t sleep a lot, maybe less than 3-4 hours ? Depend if i can sleep in my house or not.
Lucky number: No idea.
Last thing I googled: Some old french words, like “Vacuole”, because french is a rad langage and even if i speak fluent french, i love learning old slang.
Favourite fictional character: I don’t know, sadly, i don’t read a lot…
Favourite famous person: Do Lovecraft count ?
Favourite books: The Call of Cthulhu, and The Necronomicon. 
Favourite musicians: Parov Stelar. I fucking love Electroswing.
Favourite Beverage: … Well, vodka maybe …? 
Favourite Food: Chocolate mousse, and cinnamon roll.
Last movie I saw in theatres: Inside Out. 
Dream Trip: If i’m with my brother and/or with my friends, i don’t realy care, as long i’m far away from that asshole who’s my biological father.
Dream Job: Forensic scientist.
What I am wearing right now: Underwear and a black hoodie.
Dream Wedding: I don’t think i want to get married. But, something really cozy, just with close friends and family….

Activation of the Unfolded Protein Response in Sporadic Inclusion-Body Myositis but Not in Hereditary GNE Inclusion-Body Myopathy.

PubMed:
Related Articles

Activation of the Unfolded Protein Response in Sporadic Inclusion-Body Myositis but Not in Hereditary GNE Inclusion-Body Myopathy.

J Neuropathol Exp Neurol. 2015 Jun;74(6):538-46

Authors: Nogalska A, D'Agostino C, Engel WK, Cacciottolo M, Asada S, Mori K, Askanas V

Abstract
Muscle fibers in patients with sporadic inclusion-body myositis (s-IBM),the most common age-associated myopathy, are characterized by autophagic vacuoles and accumulation of ubiquitinated and congophilic multiprotein aggregates that contain amyloid-β and phosphorylated tau. Muscle fibers of autosomal-recessive hereditary inclusion-body myopathy caused by the GNE mutation (GNE-h-IBM) display similar pathologic features, except with less pronounced congophilia. Accumulation of unfolded/misfolded proteins inside the endoplasmic reticulum (ER) lumen leads to ER stress, which elicits the unfolded protein response (UPR) as a protective mechanism. Here we demonstrate for the first time that UPR is activated in s-IBM muscle biopsies, since there was 1) increased activating transcription factor 4 (ATF4) protein and increased mRNA of its target C/EBP homologous protein; 2) cleavage of the ATF6 and increased mRNA of its target glucose-regulated protein 78; and 3) an increase of the spliced form of X-box binding protein 1 and increased mRNA of ER degradation-enhancing α-mannosidase-like protein, target of heterodimer of cleaved ATF6 and spliced X-box binding protein 1. In contrast, we did not find similar evidence of the UPR induction in GNE-h-IBM patient muscle, suggesting that different intracellular mechanisms might lead to similar pathologic phenotypes. Interestingly, cultured GNE-h-IBM muscle fibers had a robust UPR response to experimental ER stress stimuli, suggesting that the GNE mutation per se is not responsible for the lack of UPR in GNE-h-IBM biopsied muscle.

PMID: 25978849 [PubMed - indexed for MEDLINE] http://dlvr.it/BgSgsh