Peripheral Neuropathy Associated with Dietary Riboflavin Deficiency in the Chicken I. Light Microscopic Study

1988 ◽  
Vol 25 (1) ◽  
pp. 9-16 ◽  
Author(s):  
W. D. Johnson ◽  
R. W. Storts
Keyword(s):  
Peripheral Neuropathy ◽  
Schwann Cell ◽  
Cell Swelling ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Tissue Separation ◽  
Leukocytic Infiltration ◽  
Leg Weakness ◽  
Demyelinating Peripheral Neuropathy ◽  
Riboflavin Deficient

Chickens fed a riboflavin-deficient diet from hatching had leg weakness and paralysis as early as 12 days of age. Signs worsened through day 16; after 35 days, recovery was evident. Sciatic nerves from affected chickens were enlarged. Significant microscopic lesions were confined to peripheral nerves and included tissue separation (suggesting interstitial edema), Schwann cell swelling, perivascular leukocytic infiltration, and segmental demyelination accompanied by accumulation of osmiophilic debris in Schwann cell cytoplasm. Axon degeneration was present, but was not a primary lesion. Acid phosphatase enzyme activity of Schwann cells was increased in affected nerves. These results demonstrate that dietary riboflavin deficiency causes a demyelinating peripheral neuropathy in young, rapidly growing chickens.

Effect of Riboflavin Deficiency on the Metabolism of Oxypurines in Chicks

1971 ◽  
Vol 49 (12) ◽  
pp. 1059-1062 ◽  
Author(s):  
S. T. Chou
Keyword(s):  
Uric Acid ◽  
Acid Synthesis ◽  
Xanthine Dehydrogenase ◽  
Broiler Chicks ◽  
Uric Acid Concentration ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
High Incidence ◽  
Liver And Kidney ◽  
Riboflavin Deficient

Day-old broiler chicks of both sexes were used in three experiments to determine the effect of riboflavin deficiency on oxypurine metabolism catalyzed by xanthine dehydrogenase, a riboflavin-containing enzyme. Chicks fed a riboflavin-deficient diet (1.38 mg/kg) for 3 weeks exhibited depressed growth and a high incidence of curled-toe paralysis (higher than 80%) as compared to control chicks (15.1 mg riboflavin per kilogram diet; no incidence of curled-toe paralysis). In addition, the precursors of uric acid, hypoxanthine and/or xanthine, accumulated in the liver and kidney of deficient chicks showing curled-toe paralysis. These observations show that dietary riboflavin being incorporated into xanthine dehydrogenase is essential for oxypurine metabolism. Moreover in the chick, the liver and the kidney may be important sites of uric acid synthesis. The low uric acid concentration in the plasma of the deficient chicks appeared to be indicative of a disturbance in uric acid synthesis in the liver and kidney.

scholarly journals Refection in rats fed on a sucrose-based, riboflavin-deficient diet

1980 ◽  
Vol 43 (1) ◽  
pp. 171-177 ◽  
Author(s):  
A. M. Prentice ◽  
C. J. Bates
Keyword(s):  
Biochemical Tests ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Biochemical Effects ◽  
Severe Deficiency ◽  
Riboflavin Deficient ◽  
Riboflavin Status

1. Refection, resulting in an increased supply of riboflavin to riboflavin-deficient rats through coprophagy, was demonstrated on a sucrose-based diet when sensitive biochemical tests of riboflavin status were employed: these included measurements of NAD(P)H2:glutathione oxidoreductase (EC 1.6.4.2); succinate:(acceptor) oxidoreductase (EC 1.3.99.1) and NADH:(acceptor) oxidoreductase (EC 1.6.99.3).2. The use of tail-cups to eliminate coprophagy, and hence refection, resulted in a more rapid and reproducible progress into severe deficiency.3. The occurrence of refection on a sucrose-based diet may account for hitherto unexplained differences between previous publications on the biochemical effects of riboflavin deficiency.

scholarly journals Effects of riboflavin deficiency and clofibrate treatment on the five acyl-CoA dehydrogenases in rat liver mitochondria

1988 ◽  
Vol 254 (2) ◽  
pp. 477-481 ◽  
Author(s):  
K Veitch ◽  
J P Draye ◽  
F Van Hoof ◽  
H S A Sherratt
Keyword(s):  
Liver Mitochondria ◽  
Rat Liver Mitochondria ◽  
Straight Chain ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Mitochondrial Fractions ◽  
Chain Acyl ◽  
Branched Chain ◽  
Acyl Coa Dehydrogenases ◽  
Riboflavin Deficient

Rats were maintained on a riboflavin-deficient diet or on a diet containing clofibrate (0.5%, w/w). The activities of the mitochondrial FAD-dependent straight-chain acyl-CoA dehydrogenases (butyryl-CoA, octanoyl-CoA and palmitoyl-CoA) and the branched-chain acyl-CoA dehydrogenases (isovaleryl-CoA and isobutyryl-CoA) involved in the degradation of branched-chain acyl-CoA esters derived from branched-chain amino acids were assayed in liver mitochondrial extracts prepared in the absence and presence of exogenous FAD. These activities were low in livers from riboflavin-deficient rats (11, 28, 16, 6 and less than 2% of controls respectively) when prepared in the absence of exogenous FAD, and were not restored to control values when prepared in 25 microM-FAD (29, 47, 28, 7 and 17%). Clofibrate feeding increased the activities of butyryl-CoA, octanoyl-CoA and palmitoyl-CoA dehydrogenases (by 48, 116 and 98% of controls respectively), but not, by contrast, the activities of isovaleryl-CoA and isobutyryl-CoA dehydrogenases (62 and 102% of controls respectively). The mitochondrial fractions from riboflavin-deficient and from clofibrate-fed rats oxidized palmitoylcarnitine in State 3 at rates of 32 and 163% respectively of those from control rats.

scholarly journals Prohibitin 1 is essential to preserve mitochondria and myelin integrity in Schwann cells

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Gustavo Della-Flora Nunes ◽  
Emma R. Wilson ◽  
Leandro N. Marziali ◽  
Edward Hurley ◽  
Nicholas Silvestri ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Schwann Cell ◽  
Schwann Cells ◽  
Peripheral Nerves ◽  
Mitochondrial Protein ◽  
Mitochondrial Damage ◽  
Homologous Protein ◽  
Demyelinating Peripheral Neuropathy ◽  
Prohibitin 1 ◽  
Cell Interface

AbstractIn peripheral nerves, Schwann cells form myelin and provide trophic support to axons. We previously showed that the mitochondrial protein prohibitin 2 can localize to the axon-Schwann-cell interface and is required for developmental myelination. Whether the homologous protein prohibitin 1 has a similar role, and whether prohibitins also play important roles in Schwann cell mitochondria is unknown. Here, we show that deletion of prohibitin 1 in Schwann cells minimally perturbs development, but later triggers a severe demyelinating peripheral neuropathy. Moreover, mitochondria are heavily affected by ablation of prohibitin 1 and demyelination occurs preferentially in cells with apparent mitochondrial loss. Furthermore, in response to mitochondrial damage, Schwann cells trigger the integrated stress response, but, contrary to what was previously suggested, this response is not detrimental in this context. These results identify a role for prohibitin 1 in myelin integrity and advance our understanding about the Schwann cell response to mitochondrial damage.

STUDIES OF THE MOLECULAR BASIS OF CONGENITAL MALFORMATIONS

PEDIATRICS ◽  
1969 ◽  
Vol 43 (6) ◽  
pp. 915-926
Author(s):  
Bruce Mackler
Keyword(s):  
Congenital Malformations ◽  
Transport Systems ◽  
Congenital Defects ◽  
Synthetic Analog ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Adult Rats ◽  
Prenatal Environment ◽  
Better Regulation ◽  
Riboflavin Deficient

With recent advances in treatment of many acute and chronic diseases, the problems associated with congenital malformations in children have assumed a greater importance in pediatrics. Previous to the work of Warkany and Nelson in 19401, it was recognized that many congenital defects were genetically determined and hereditary. The finding by Warkany and co-workers1-3 that modification of the prenatal environment by severe states of riboflavin deficiency produced congenital malformations in mammals demonstrated clearly the importance of the prenatal environment in the development of the fetus and gave impetus to searches for other environmental factors of importance to the developing organism. The syndrome resulting from severe maternal riboflavin deficiency in rats is characterized mainly by micrognathia and reduction type of defects in the distal extremities of the fetuses, and by a wide number of other anomalies such as cleft palate and hydronephrosis. More recently, Nelson and co-workers4 demonstrated that galactoflavin, a synthetic analog of riboflavin, produced a rapid riboflavin deficiency syndrome in pregnant rats. The addition of galactoflavin to a riboflavin-deficient diet materially shortened the time required to produce riboflavin deficiency, and permitted much better regulation of the experimental model. In other studies of riboflavin deficiency, Miller and co-workers5 showed a lower flavin content in fetuses from riboflavindeficient rats than in control fetuses, suggesting the possibility that there may be corresponding deficiencies in the activities of flavin-dependent enzymes such as the terminal electron transport systems (ETP). Other investigators6 have reported studies of enzymatic activities in liver mitochondria of adult rats fed a riboflavin-deficient diet with added galactoflavin, but with varying results.

scholarly journals The Anemia of Human Riboflavin Deficiency

Blood ◽  
1965 ◽  
Vol 25 (4) ◽  
pp. 432-442 ◽  
Author(s):  
MONTAGUE LANE ◽  
CLARENCE P. ALFREY
Keyword(s):  
Bone Marrow ◽  
Normocytic Anemia ◽  
Adult Males ◽  
Normal Range ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Platelet Counts ◽  
Leukocyte Counts ◽  
Riboflavin Deficient

Abstract 1. Riboflavin deficiency was induced in 8 adult males with a riboflavin deficient diet and a riboflavin antagonist, galactoflavin. 2. Each patient developed a normochromic normocytic anemia and reticulocytopenia. 3. The leukocyte counts and platelet counts remained within the normal range throughout the period of deficiency. 4. The bone marrow changes and ferrokinetic characteristics of this anemia have been described. 5. The anemia was reversed by riboflavin administration.

SOME EFFECTS OF A MATERNAL RIBOFLAVIN DEFICIENCY ON REPRODUCTION IN THE RAT

1952 ◽  
Vol 30 (5) ◽  
pp. 383-389 ◽  
Author(s):  
J. P. W. Gilman ◽  
F. Armstrong Perry ◽  
D. C. Hill
Keyword(s):  
Food Consumption ◽  
Albino Rats ◽  
Corpora Lutea ◽  
Vaginal Smears ◽  
Riboflavin Deficiency ◽  
Deficient Diet ◽  
Alizarin Red ◽  
Implantation Sites ◽  
Riboflavin Deficient ◽  
Ossification Centers

Ten female albino rats were fed a purified riboflavin deficient diet for periods of 17 to 28 days before breeding and throughout pregnancy. Vaginal smears were taken daily throughout this period, and food consumption and weight gains were recorded. All animals were sacrificed 21 days after breeding and examined for viable young, fetal remnants, implantation sites, and corpora lutea. Living young were examined both macroscopically and by the alizarin red S technique for ossification centers. Five of these females produced 24 viable fetuses, of which two showed skeletal anomalies, one was oedematous, and the remainder exhibited a significant weight reduction. An average of 62.5% of the embryos in these litters were undergoing resorption. The remaining five deficient mothers exhibited 100% resorptions. Nine control females, treated similarly to the above except for the addition of riboflavin to their diet, had an average of 10 living fetuses and 14.1% resorptions per litter with no abnormals. The erythrocyte sign ranged from complete absence in controls with no resorbing fetuses to a duration of up to six days in some depleted animals, with a correlation of +0.84 between duration and percentage of resorptions.

scholarly journals mTORC1 and JUN are activated after deletion of Prohibitin 1 in Schwann cells and may link mitochondrial dysfunction to demyelination

2020 ◽  
Author(s):  
Gustavo Della-Flora Nunes ◽  
Emma R. Wilson ◽  
Edward Hurley ◽  
Bin He ◽  
Bert W. O’Malley ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Schwann Cell ◽  
Mitochondrial Dysfunction ◽  
Schwann Cells ◽  
Critical Role ◽  
Mitochondrial Protein ◽  
Important Regulator ◽  
Demyelinating Peripheral Neuropathy ◽  
Prohibitin 1

AbstractSchwann cell (SC) mitochondria are quickly emerging as an important regulator of myelin maintenance in the peripheral nervous system (PNS). However, the mechanisms underlying demyelination in the context of mitochondrial dysfunction in the PNS are incompletely understood. We recently showed that conditional ablation of the mitochondrial protein Prohibitin 1 (Phb1) in SCs causes a severe and fast progressing demyelinating peripheral neuropathy, but the mechanism that causes failure of myelin maintenance remained unknown. Here, we report that mTORC1 and JUN are continuously activated in the absence of Phb1, likely due to mitochondrial damage. Moreover, we demonstrate that these pathways are involved in the demyelination process, and that inhibition of mTORC1 using rapamycin partially rescues the demyelinating pathology. Therefore, we propose that mTORC1 and JUN may play a critical role as executioners of demyelination in the context of perturbations to SC mitochondria.

scholarly journals Activation of mTORC1 and c-Jun by Prohibitin1 loss in Schwann cells may link mitochondrial dysfunction to demyelination

eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Gustavo Della Flora Nunes ◽  
Emma R Wilson ◽  
Edward Hurley ◽  
Bin He ◽  
Bert W O'Malley ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Schwann Cell ◽  
Mitochondrial Dysfunction ◽  
Schwann Cells ◽  
Critical Role ◽  
Mitochondrial Protein ◽  
Important Regulator ◽  
Demyelinating Peripheral Neuropathy ◽  
Prohibitin 1

Schwann cell (SC) mitochondria are quickly emerging as an important regulator of myelin maintenance in the peripheral nervous system (PNS). However, the mechanisms underlying demyelination in the context of mitochondrial dysfunction in the PNS are incompletely understood. We recently showed that conditional ablation of the mitochondrial protein Prohibitin 1 (PHB1) in SCs causes a severe and fast progressing demyelinating peripheral neuropathy in mice, but the mechanism that causes failure of myelin maintenance remained unknown. Here, we report that mTORC1 and c-Jun are continuously activated in the absence of Phb1, likely as part of the SC response to mitochondrial damage. Moreover, we demonstrate that these pathways are involved in the demyelination process, and that inhibition of mTORC1 using rapamycin partially rescues the demyelinating pathology. Therefore, we propose that mTORC1 and c-Jun may play a critical role as executioners of demyelination in the context of perturbations to SC mitochondria.

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