Berlit, Peter

Bonjour, Jean-Pierre

Krause, Klaus-Henning

English

Universität München: Elektronischen Publikationen

4C 
Band54 1984/ ( 
International Journal for Vitamin and Nutrition Research 
Internationale Zeitschrift 
für Vitamin- und Ernährungsforschung 
Journal international de Vitaminologie et de Nutrition 
E D I T O R - I N - C H I E F 
G. R I T Z E L , ST. A L B A N V O R S T A D T 19, CH-4052 B A S E L 
C U R A T O R E S 
K.Bernhard, Basel R.S.Harris, Cambridge, Mass. R.E. Stone, Chicago 
H . D . Cremer, Glessen C.den Hartog, Wageningen A . V . Szent-Györgyi, Woods Hole 
K. Folkers, Austin T. Reichstein, Basel 
E D I T O R E S 
J.T. Abrams, Cambridge, GB 
H . Berger, Innsbruck 
H . Bickel, Zürich 
A . Bondi, Rehovoth 
M . Br in , Nutley 
G. Brubacher, Basel 
R. Buzina, Zagreb 
D.B. Coursin, Lancaster 
P . M . Dreyfus, Sacramento 
F. Fidanza, Perugia 
J.Ganguly, Bangalore 
J. Mauron, La Tour-de-Peilz 
H . E . Sauberlich, Birmingham, USA 
M . L . Scott, Ithaca 
The "International Journal for Vitamin and Nutri t ion Research" is published at irregular intervals 
(about 4 fasc. per annum) 4 fasc. = 1 volume, prize per volume = Sw.Fr. 134.— / Die «Internat ionale 
Zeitschrift für Vitamin- und Ernährungsforschung» erscheint in zwangloser Reihenfolge (ca. 4 Hefte pro 
Jahr). 4 Hefte ergeben einen Band. Bezugspreis pro Band= sFr. 134.—, D M 170.— / Le «Journa l inter-
national de Vitaminologie et de Nutr i t ion» parait irregulierement en fascicules (env. 4 fasc. par annee). 
4 fasc. = 1 volume, prix par volume = sfr. 134.— 
Hans Huber Publishers Berne Stuttgart Toronto 
Western Hemisphere: Jack K. Burgess Inc., 2175 Lemoine Avenue, Fort Lee, N . J . 07024 
This Journal is regularly listed in Current Contents and Index Medicus 
The Journal serves as the official organ of the Swiss Society for Nut r i t ion Research 
Printed in Switzerland © 1984 by Hans Huber Publishers, Berne 
Inhalt / Contents 
A R A F A A . M . and HUSSEIN L . : The Assessment o f The Vitamin B 6 Status among Egyptian School 
Children by Measuring the Urinary Cystathionine Excretion 321 
A R I M A N A N A L . and L E A T H W O O D P.D.: Effects of Prior Carbohydrate Intake on Protein/Carbo­
hydrate Selection by the Rat 283 
A R N A U D M . J . , WELSCH C , GARREL D. and TOURNIAIRE J.: Reproducibility of the Measurement 
of Whole Body Protein Turnover Using the End Product Method 282 
BASU T . K . , WEISER, Τ . and DEMPSTER J.F.: A n in vitro Effect of Ascorbate on the Spontaneous 
Reduction of Sodium Nitrite Concentration in a Reaction Mixture 233 
BAYLISS R . M . , BROOKES R., M C C U L L O C H J., K U Y L J . M . and M E T Z J.: Urinary Thiamine Excretion 
after Oral Physiological Doses o f the Vitamin 161 
B I C K E L R., REINHARDT C.A. and L Ü T H Y J.: In vitro Growth Inhibition Assay for the Detection of 
Fusarium Toxins in Feedstuffs 287 
BIESALSKI H . K . : Retinol and Retinyl Ester in Separated Structures of the Guinea Pig Inner Ear . . . 113 
B L U M M . , P R A B U C K I A . L . and SCHÜRCH Α . : The Influence of Different Dietary Fats on the Life 
Span of Rats 276 
B L U M M . , PRABUCKI A . L . and SCHÜRCH Α . : The Influence of Various Dietary Fats on the Fatty 
Acid Composition o f Organ Lipids in the Rat 277 
BONJOUR J.-P., BAUSCH J., SUORMALA T. and BAUMGARTNER E.R.: Detection of Biocytin in Urine 
of Children with Congenital Biotinidase Deficiency 223 
BOUCHER-EHRENSPERGER M . and PETTER C : Prevention of Thrombocytic Defects in the br/br 
Rabbit with Folic acid and Vitamin B l 2 : Analogy with the T .A .R . Syndrome in Humans 199 
BRANCA D . , SCUTARI G. and SILIPRANDI N . : Pantethine and Pantothenate Effect on the CoA Con­
tent o f Rat Liver 211 
CASTER W.O. , D R U M M O N D S.K. and T A N N E R M . A . : Maternal Diet and Mental Retardation in 
Southern Georgia, USA 371 
CHANGBUMRUNG S., POSHAKRISHANA P., V U D H I V A I N . , HONGTONG K. , PONGPAEW P., MIGASENA 
P. et al: Measurements of B , , B 2 , B 6 Status in Children and their Mothers Attenting a Well-
baby Clinic in Bangkok 149 
C U L L U M M . E . , OLSON J.A. and VEYSEY S.: Analysis of Deuterated Analogs of Vitamin A by Elec­
tron Impact and Chemical Ionization Modes in Gas Chromatography Coupled to Mass Spec­
trometry 3 
C U L L U M M . E . , JOHNSON C. and Z I L E M . H . : Comparison of Fetal and Adult Retinol and Retinoic 
Acid Binding Proteins in Bovine Serum and Pigment Epithelium 297 
C U L L U M M . E . , Z I L E M . H . and VEYSEY S.W.: Analysis of Retinol and Dideuterated Retinol in Rat 
Plasma by Gas Chromatography Combined Mass Spectrometry 11 
DECOMBAZ J., SARTORI D . , A R N A U D A . L . , T H E L I N A . L . , SCHÜRCH P. and H O W A L D H . : Oxidation 
and Metabolic Effects of Fructose or Glucose Ingested before Exercise in Man 281 
DOREA J .G. , SOUZA J .Α . , G A L V A O Μ . Ο . and IUNES Μ . Α . F . : Concentration of Vitamin A in the 
Liver of Foetuses and Infants dying of Various Causes in Brasilia, Brazil 119 
F I D A N Z A A . A . : Nutritional Status of the Elderly, IV 361 
F I D A N Z A F., BRUBACHER G., SIMONETTI M.S. and C U C C H I A L . M . : Nutritional Status of the Eld­
erly, I I I 355 
F I D A N Z A F., F I D A N Z A A . A . , C O L I R. and M E N C A R I N I C.A.: Food and Nutritient Consumption of 
Two Rural Italian Population Groups Followed for Twenty Years 91 
F I D A N Z A F., SIMONETTI M.S. , C U C C H I A L . M . , BALUCCA G.G. and LOSITO G.: Nutritional Status of 
the Elderly, I I 75 
FISHBAINE B. and BUTTERFIELD G.: Ascorbic Acid Status of Running and Sedentary Men 273 
F L A T T J.P., ACHESON K . J . , RAVUSSIN E. and JEQUIER E.: Dietary Fat Substrate Utilization and 
Short Term Body Composition Changes in Man 279 
F L O R I D I Α. , PUPITA M . , P A L M E R I N I C A . , F I N I C. and F I D A N Z A A . A . : Thiamin Pyrophosphate De­
termination in Whole Blood and Erythrocytes by High Performance Liquid Chromatography . 165 
FRIGG M . and BROZ J.: Relationships between Vitamin A and Vitamin Ε in the Chick 125 
GIBSON J . and WESTHUYZEN J . V A N DER: Effect o f L-Dihydroxyphenylalanine (L-dopa) and Met­
hionine on Tissue S-Adenosylmethionine Concentrations in Cobalamin-inactivated Fruit Bats . 329 
G L E N N B . P . and E L Y O.G.: Effect o f Lipid-coated Lysine on Digestion and Nitrogen Metabolism 
by Wethers 377 
GRECO A . M . , BOSCHI G. , STICCHI R., V E T R A N I A . and SALVATORE G.: Effects of a Purified Diet 
Enriched with Animal Protein on Female Rats. Preliminary Report 263 
G U I D O U X R.: C a 2 + Regulation by Isolated Rat Liver Mitochondria Influence of the Intramito­
chondrial Citrate Content on the Extramitochondrial C a 2 + Level 288 
GUIGOZ Y . and JUILLERAT M . : Induction of Tyrosine Aminotransferase (TAT) by a Physiological 
Stress as Function of Dietary Fat 289 
G U I L L A N D J.C., BEREKSI-REGUIG B. , L E Q U E U B. , M O R E A U D. and KLEPPING J.: Evaluation of Py-
ridoxine Intake and Pyridoxine Status among Aged Institutionalised People 185 
HIDIROGLOU M . and W I L L I A M S C.J.: Vitamin D 3 Levels in Certain Sheep Tissues at Various Times 
after the Intramuscular Administration of Vitamin D 3 17 
H O L L O W A Y D.E. , G U I R Y V.C. , H O L L O W AY B .A. and RIVERS J . M . : Influence of Dietary Ascorbic 
Acid on Cholesterol 7-a-Hydroxylase Activity in the Rat 333 
H O L M J. , H A N S E N I . and LYNGBYE J.: Quantitative and Qualitative Effects of N 1 0 -Methylfolate on 
High-affinity Folate Binding in Human Leukocytes 195 
H O R I U C H I S. and Ο Ν Ο S.: Effects of Riboflavin Administration on the Phospholipid Metabolism 
of Rat Liver Impaired with Carbon Tetrachloride 173 
H U N T C , CHAKRAVORTY N . K . and A N N A N G.: The Clinical and Biochemical Effects of Vitamin C 
Supplementation in Short-stay Hospitalized Geriatric Patients 65 
KIRCHGESSNER M . and M Ü L L E R H . L . : Thermogenesis with a Ketogenic Diet in Sows 99 
KORPELA H . , LOUENIVA R., Y R Ä N H E I K K I Ε. and KAUPPILA Α.: Selenium Concentration in Mater­
nal and Umbilical Cord Blood, Placenta and Amniotic Membranes 257 
K R A T Z I N G C.C., KELLY J.D. and OELRICHS B.A. : Ascorbic Acid Changes in Brain 349 
KRAUS K . - H . , BONJOUR, J.-P. and BERLIT P.: Biotin Levels in Plasma of Patients with Friedreich 
Ataxia and Other Spinocerebellar Degenerations 272 
KRAUSE K . - H . , K O C H E N W. , BERLIT P. and BONJOUR J.-P.: Excretion of Organic Acids Associated 
with Biotin Deficiency in Chronic Anticonvulsant Therapy 217 
K U M P U L A I N E N J., V U O R I E. and STIMES M . A . : Effect o f Maternal Dietary Selenium Intake on Sele­
nium Levels in Breast Mi lk 251 
K U N Z C , NIESEN Μ. , LILIENFELD-TOAL L . VON, and BURMEISTER W.: Vitamin D , 25-Hydroxy-
Vitamin D and 1,25-Dihydroxy-Vitamin D in Cow's M i l k , Infant Formulas and Breast Mi lk 
during Different Stages of Lactation 141 
LABADARIOS D . , SHEPHARD G.S., M I N E U R L . G . , VAN BUUREN A . J . , HUTCHISON M . E . and OOST-
HUIZEN O.J.: Biochemical Vitamin B 6 Deficiency in Adults with Chronic Glomerulonephrides 
with and without the Nephrotic Syndrome 313 
LANGHANS W. and SCHARRER E.: Feeding Rats a High Fat Diet Changes the Effect of Various Me­
tabolic Challenges on Food Intake 280 
MOSER U . and WEBER F.: Uptake of Ascorbic Acid by Human Granulocytes 47 
M O E N N O Z D. and ASHLEY D.V. : Comparison of Plasma Free Tryptophan and Plasma Tryptophan 
to Large Neutral Amino Acid Ratios as Physiological Predictors o f Brain Tryptophan 285 
M O E N N O Z D . , EXELL N . and ASHLEY D .V. : Lack of Influence of Free Fatty Acids on Tryptophan-
albumin Binding in Plasma 286 
NADIGER H . A . , R A D A.S. and SADASIVUDU B. : Effect of Simultaneous Administration o f Vitamin 
Ε and Pyridoxin on Erythrocyte Membrane Na + K~ATPase Activity 307 
OELRICHS B .A . , K R A T Z I N G C.C., K E L L Y J.D. and W I N Z O R D.J . : The Binding of Ascorbate to Bovi­
ne Serum Albumin 61 
PIIRONEN V. , V A R O P., SYVÄOJA E. -L . , SALMINEN K. , KOIVISTOINEN P. and A R V I L O M M I H . : High 
Performance Liquid Chromatographic Determination of Tocopherols and Tocotrienols and its 
Application to Diets and Plasma of Finnish Men, I I 41 
PIIRONEN V . , V A R O P., SYVÄOJA E. -L. , SALMINEN K. and KOIVISTOINEN P.: High Performance L i -
quid Chromatographic Determination o f Tocopherols and Tocotrienols and its Application to 
Diets and Plasma of Finnish Men, 1 35 
272 
Research Note 
Biotin Levels in Plasma of Patients with Friedreich Ataxia and Other Spinocerebellar Degenerations 
K L A U S - H E N N I N G K R A U S E 1 , JEAN-PIERRE BONJOUR 2 and PETER BERLIT 1 
In patients with Friedreich ataxia low pyruvate carboxylase (PC) activity in liver and fibroblasts has 
been described recently [ 1 ] . PC activity can be lowered due to reduced circulating biotin levels caused by 
disturbances of the metabolism, absorption or transport of biotin. We determined plasma biotin levels 
using Lactobacillus plantarum as test organism [ 2 ] , in 1 2 patients with spinocerebellar disorders, and 
compared the concentrations with those o f normal controls, matched for age ( ± 3 ys) and sex. 5 non-
related patients (cases 1-5) exhibited the typical characteristics of Friedreich ataxia with recessive familial 
involvement, 2 patients (cases 6 and 7 ) showed a sporadic olivopontocerebellar atrophy, 3 siblings (cases 
8 - 1 0 ) a combination of cerebellar ataxia with degeneration of anterior column cells, and 2 siblings (cases 
1 1 and 12) cerebellar atrophy of Nonne-Pierre-Marie type. As shown in the table, the patients with 
Friedreich ataxia did not have low biotin plasma levels; this finding indicates, that the reduced PC activi-
ty observed in Friedreich ataxia is not due to a decrease in biotin level. In contrast to all other patients, 
the two patients with sporadic olivopontocerebellar atrophy showed low biotin levels o f 188 and 185 ng/ I . 
The clinical implication of this finding is not clear. Oral medication with biotin in a dose of 1 0 mg per day-
over a period of three months showed no certain improvement of the disorder in patients 6 and 7 . 
Table I : Biotin levels in plasma (ng/1) in patients with spinocerebellar disorders and in controls 
No of patient biotin in plasma 
of patients (ng/I) 
biotin in plasma 
of controls (ng/I) 
1 3 4 8 373 
2 3 2 2 3 6 7 
3 347 263 
4 3 1 7 435 
5 588 3 4 8 
6 188 3 8 2 
7 185 3 5 7 
8 3 3 2 243 
9 3 1 7 343 
1 0 2 4 7 338 
11 3 0 0 375 
1 2 3 1 2 345 
References: 1. DIJKSTRA, U . J . , W I L L A N S , J . L . , JOOSTEN, E . M . G . , GABRIEELS, F . J . M . : Ann. Neurol. 
13, 325 ( 1 9 8 3 ) . - 2 . FRIGG, M . , BRUBACHER, G . : Internat. J . Vi t . Nutr. Res. 46, 3 1 4 ( 1 9 7 6 ) . 
1 Neurologische Universitätsklinik, Voss-Str. 2 , D - 6 9 0 0 Heidelberg, F.R.G. 
2 Department of Vitamin and Nutri t ion Research, F. Hoffmann-La Roche & Co., Basel, Switzerland. 


Biotin levels in plasma of patients with Friedreich ataxia and other spinocerebellar degenerations

Open Access LMU

4C Band54 1984/ ( International Journal for Vitamin and Nutrition Research Internationale Zeitschrift für Vitamin- und Ernährungsforschung Journal international de Vitaminologie et de Nutrition E D I T O R - I N - C H I E F G. R I T Z E L , ST. A L B A N V O R S T A D T 19, CH-4052 B A S E L C U R A T O R E S K.Bernhard, Basel R.S.Harris, Cambridge, Mass. R.E. Stone, Chicago H . D . Cremer, Glessen C.den Hartog, Wageningen A . V . Szent-Györgyi, Woods Hole K. Folkers, Austin T. Reichstein, Basel E D I T O R E S J.T. Abrams, Cambridge, GB H . Berger, Innsbruck H . Bickel, Zürich A . Bondi, Rehovoth M . Br in , Nutley G. Brubacher, Basel R. Buzina, Zagreb D.B. Coursin, Lancaster P . M . Dreyfus, Sacramento F. Fidanza, Perugia J.Ganguly, Bangalore J. Mauron, La Tour-de-Peilz H . E . Sauberlich, Birmingham, USA M . L . Scott, Ithaca The "International Journal for Vitamin and Nutri t ion Research" is published at irregular intervals (about 4 fasc. per annum) 4 fasc. = 1 volume, prize per volume = Sw.Fr. 134.— / Die «Internat ionale Zeitschrift für Vitamin- und Ernährungsforschung» erscheint in zwangloser Reihenfolge (ca. 4 Hefte pro Jahr). 4 Hefte ergeben einen Band. Bezugspreis pro Band= sFr. 134.—, D M 170.— / Le «Journa l inter-national de Vitaminologie et de Nutr i t ion» parait irregulierement en fascicules (env. 4 fasc. par annee). 4 fasc. = 1 volume, prix par volume = sfr. 134.— Hans Huber Publishers Berne Stuttgart Toronto Western Hemisphere: Jack K. Burgess Inc., 2175 Lemoine Avenue, Fort Lee, N . J . 07024 This Journal is regularly listed in Current Contents and Index Medicus The Journal serves as the official organ of the Swiss Society for Nut r i t ion Research Printed in Switzerland © 1984 by Hans Huber Publishers, Berne Inhalt / Contents A R A F A A . M . and HUSSEIN L . : The Assessment o f The Vitamin B 6 Status among Egyptian School Children by Measuring the Urinary Cystathionine Excretion 321 A R I M A N A N A L . and L E A T H W O O D P.D.: Effects of Prior Carbohydrate Intake on Protein/Carbo­hydrate Selection by the Rat 283 A R N A U D M . J . , WELSCH C , GARREL D. and TOURNIAIRE J.: Reproducibility of the Measurement of Whole Body Protein Turnover Using the End Product Method 282 BASU T . K . , WEISER, Τ . and DEMPSTER J.F.: A n in vitro Effect of Ascorbate on the Spontaneous Reduction of Sodium Nitrite Concentration in a Reaction Mixture 233 BAYLISS R . M . , BROOKES R., M C C U L L O C H J., K U Y L J . M . and M E T Z J.: Urinary Thiamine Excretion after Oral Physiological Doses o f the Vitamin 161 B I C K E L R., REINHARDT C.A. and L Ü T H Y J.: In vitro Growth Inhibition Assay for the Detection of Fusarium Toxins in Feedstuffs 287 BIESALSKI H . K . : Retinol and Retinyl Ester in Separated Structures of the Guinea Pig Inner Ear . . . 113 B L U M M . , P R A B U C K I A . L . and SCHÜRCH Α . : The Influence of Different Dietary Fats on the Life Span of Rats 276 B L U M M . , PRABUCKI A . L . and SCHÜRCH Α . : The Influence of Various Dietary Fats on the Fatty Acid Composition o f Organ Lipids in the Rat 277 BONJOUR J.-P., BAUSCH J., SUORMALA T. and BAUMGARTNER E.R.: Detection of Biocytin in Urine of Children with Congenital Biotinidase Deficiency 223 BOUCHER-EHRENSPERGER M . and PETTER C : Prevention of Thrombocytic Defects in the br/br Rabbit with Folic acid and Vitamin B l 2 : Analogy with the T .A .R . Syndrome in Humans 199 BRANCA D . , SCUTARI G. and SILIPRANDI N . : Pantethine and Pantothenate Effect on the CoA Con­tent o f Rat Liver 211 CASTER W.O. , D R U M M O N D S.K. and T A N N E R M . A . : Maternal Diet and Mental Retardation in Southern Georgia, USA 371 CHANGBUMRUNG S., POSHAKRISHANA P., V U D H I V A I N . , HONGTONG K. , PONGPAEW P., MIGASENA P. et al: Measurements of B , , B 2 , B 6 Status in Children and their Mothers Attenting a Well-baby Clinic in Bangkok 149 C U L L U M M . E . , OLSON J.A. and VEYSEY S.: Analysis of Deuterated Analogs of Vitamin A by Elec­tron Impact and Chemical Ionization Modes in Gas Chromatography Coupled to Mass Spec­trometry 3 C U L L U M M . E . , JOHNSON C. and Z I L E M . H . : Comparison of Fetal and Adult Retinol and Retinoic Acid Binding Proteins in Bovine Serum and Pigment Epithelium 297 C U L L U M M . E . , Z I L E M . H . and VEYSEY S.W.: Analysis of Retinol and Dideuterated Retinol in Rat Plasma by Gas Chromatography Combined Mass Spectrometry 11 DECOMBAZ J., SARTORI D . , A R N A U D A . L . , T H E L I N A . L . , SCHÜRCH P. and H O W A L D H . : Oxidation and Metabolic Effects of Fructose or Glucose Ingested before Exercise in Man 281 DOREA J .G. , SOUZA J .Α . , G A L V A O Μ . Ο . and IUNES Μ . Α . F . : Concentration of Vitamin A in the Liver of Foetuses and Infants dying of Various Causes in Brasilia, Brazil 119 F I D A N Z A A . A . : Nutritional Status of the Elderly, IV 361 F I D A N Z A F., BRUBACHER G., SIMONETTI M.S. and C U C C H I A L . M . : Nutritional Status of the Eld­erly, I I I 355 F I D A N Z A F., F I D A N Z A A . A . , C O L I R. and M E N C A R I N I C.A.: Food and Nutritient Consumption of Two Rural Italian Population Groups Followed for Twenty Years 91 F I D A N Z A F., SIMONETTI M.S. , C U C C H I A L . M . , BALUCCA G.G. and LOSITO G.: Nutritional Status of the Elderly, I I 75 FISHBAINE B. and BUTTERFIELD G.: Ascorbic Acid Status of Running and Sedentary Men 273 F L A T T J.P., ACHESON K . J . , RAVUSSIN E. and JEQUIER E.: Dietary Fat Substrate Utilization and Short Term Body Composition Changes in Man 279 F L O R I D I Α. , PUPITA M . , P A L M E R I N I C A . , F I N I C. and F I D A N Z A A . A . : Thiamin Pyrophosphate De­termination in Whole Blood and Erythrocytes by High Performance Liquid Chromatography . 165 FRIGG M . and BROZ J.: Relationships between Vitamin A and Vitamin Ε in the Chick 125 GIBSON J . and WESTHUYZEN J . V A N DER: Effect o f L-Dihydroxyphenylalanine (L-dopa) and Met­hionine on Tissue S-Adenosylmethionine Concentrations in Cobalamin-inactivated Fruit Bats . 329 G L E N N B . P . and E L Y O.G.: Effect o f Lipid-coated Lysine on Digestion and Nitrogen Metabolism by Wethers 377 GRECO A . M . , BOSCHI G. , STICCHI R., V E T R A N I A . and SALVATORE G.: Effects of a Purified Diet Enriched with Animal Protein on Female Rats. Preliminary Report 263 G U I D O U X R.: C a 2 + Regulation by Isolated Rat Liver Mitochondria Influence of the Intramito­chondrial Citrate Content on the Extramitochondrial C a 2 + Level 288 GUIGOZ Y . and JUILLERAT M . : Induction of Tyrosine Aminotransferase (TAT) by a Physiological Stress as Function of Dietary Fat 289 G U I L L A N D J.C., BEREKSI-REGUIG B. , L E Q U E U B. , M O R E A U D. and KLEPPING J.: Evaluation of Py-ridoxine Intake and Pyridoxine Status among Aged Institutionalised People 185 HIDIROGLOU M . and W I L L I A M S C.J.: Vitamin D 3 Levels in Certain Sheep Tissues at Various Times after the Intramuscular Administration of Vitamin D 3 17 H O L L O W A Y D.E. , G U I R Y V.C. , H O L L O W AY B .A. and RIVERS J . M . : Influence of Dietary Ascorbic Acid on Cholesterol 7-a-Hydroxylase Activity in the Rat 333 H O L M J. , H A N S E N I . and LYNGBYE J.: Quantitative and Qualitative Effects of N 1 0 -Methylfolate on High-affinity Folate Binding in Human Leukocytes 195 H O R I U C H I S. and Ο Ν Ο S.: Effects of Riboflavin Administration on the Phospholipid Metabolism of Rat Liver Impaired with Carbon Tetrachloride 173 H U N T C , CHAKRAVORTY N . K . and A N N A N G.: The Clinical and Biochemical Effects of Vitamin C Supplementation in Short-stay Hospitalized Geriatric Patients 65 KIRCHGESSNER M . and M Ü L L E R H . L . : Thermogenesis with a Ketogenic Diet in Sows 99 KORPELA H . , LOUENIVA R., Y R Ä N H E I K K I Ε. and KAUPPILA Α.: Selenium Concentration in Mater­nal and Umbilical Cord Blood, Placenta and Amniotic Membranes 257 K R A T Z I N G C.C., KELLY J.D. and OELRICHS B.A. : Ascorbic Acid Changes in Brain 349 KRAUS K . - H . , BONJOUR, J.-P. and BERLIT P.: Biotin Levels in Plasma of Patients with Friedreich Ataxia and Other Spinocerebellar Degenerations 272 KRAUSE K . - H . , K O C H E N W. , BERLIT P. and BONJOUR J.-P.: Excretion of Organic Acids Associated with Biotin Deficiency in Chronic Anticonvulsant Therapy 217 K U M P U L A I N E N J., V U O R I E. and STIMES M . A . : Effect o f Maternal Dietary Selenium Intake on Sele­nium Levels in Breast Mi lk 251 K U N Z C , NIESEN Μ. , LILIENFELD-TOAL L . VON, and BURMEISTER W.: Vitamin D , 25-Hydroxy-Vitamin D and 1,25-Dihydroxy-Vitamin D in Cow's M i l k , Infant Formulas and Breast Mi lk during Different Stages of Lactation 141 LABADARIOS D . , SHEPHARD G.S., M I N E U R L . G . , VAN BUUREN A . J . , HUTCHISON M . E . and OOST-HUIZEN O.J.: Biochemical Vitamin B 6 Deficiency in Adults with Chronic Glomerulonephrides with and without the Nephrotic Syndrome 313 LANGHANS W. and SCHARRER E.: Feeding Rats a High Fat Diet Changes the Effect of Various Me­tabolic Challenges on Food Intake 280 MOSER U . and WEBER F.: Uptake of Ascorbic Acid by Human Granulocytes 47 M O E N N O Z D. and ASHLEY D.V. : Comparison of Plasma Free Tryptophan and Plasma Tryptophan to Large Neutral Amino Acid Ratios as Physiological Predictors o f Brain Tryptophan 285 M O E N N O Z D . , EXELL N . and ASHLEY D .V. : Lack of Influence of Free Fatty Acids on Tryptophan-albumin Binding in Plasma 286 NADIGER H . A . , R A D A.S. and SADASIVUDU B. : Effect of Simultaneous Administration o f Vitamin Ε and Pyridoxin on Erythrocyte Membrane Na + K~ATPase Activity 307 OELRICHS B .A . , K R A T Z I N G C.C., K E L L Y J.D. and W I N Z O R D.J . : The Binding of Ascorbate to Bovi­ne Serum Albumin 61 PIIRONEN V. , V A R O P., SYVÄOJA E. -L . , SALMINEN K. , KOIVISTOINEN P. and A R V I L O M M I H . : High Performance Liquid Chromatographic Determination of Tocopherols and Tocotrienols and its Application to Diets and Plasma of Finnish Men, I I 41 PIIRONEN V . , V A R O P., SYVÄOJA E. -L. , SALMINEN K. and KOIVISTOINEN P.: High Performance L i -quid Chromatographic Determination o f Tocopherols and Tocotrienols and its Application to Diets and Plasma of Finnish Men, 1 35 272 Research Note Biotin Levels in Plasma of Patients with Friedreich Ataxia and Other Spinocerebellar Degenerations K L A U S - H E N N I N G K R A U S E 1 , JEAN-PIERRE BONJOUR 2 and PETER BERLIT 1 In patients with Friedreich ataxia low pyruvate carboxylase (PC) activity in liver and fibroblasts has been described recently [ 1 ] . PC activity can be lowered due to reduced circulating biotin levels caused by disturbances of the metabolism, absorption or transport of biotin. We determined plasma biotin levels using Lactobacillus plantarum as test organism [ 2 ] , in 1 2 patients with spinocerebellar disorders, and compared the concentrations with those o f normal controls, matched for age ( ± 3 ys) and sex. 5 non-related patients (cases 1-5) exhibited the typical characteristics of Friedreich ataxia with recessive familial involvement, 2 patients (cases 6 and 7 ) showed a sporadic olivopontocerebellar atrophy, 3 siblings (cases 8 - 1 0 ) a combination of cerebellar ataxia with degeneration of anterior column cells, and 2 siblings (cases 1 1 and 12) cerebellar atrophy of Nonne-Pierre-Marie type. As shown in the table, the patients with Friedreich ataxia did not have low biotin plasma levels; this finding indicates, that the reduced PC activi-ty observed in Friedreich ataxia is not due to a decrease in biotin level. In contrast to all other patients, the two patients with sporadic olivopontocerebellar atrophy showed low biotin levels o f 188 and 185 ng/ I . The clinical implication of this finding is not clear. Oral medication with biotin in a dose of 1 0 mg per day-over a period of three months showed no certain improvement of the disorder in patients 6 and 7 . Table I : Biotin levels in plasma (ng/1) in patients with spinocerebellar disorders and in controls No of patient biotin in plasma of patients (ng/I) biotin in plasma of controls (ng/I) 1 3 4 8 373 2 3 2 2 3 6 7 3 347 263 4 3 1 7 435 5 588 3 4 8 6 188 3 8 2 7 185 3 5 7 8 3 3 2 243 9 3 1 7 343 1 0 2 4 7 338 11 3 0 0 375 1 2 3 1 2 345 References: 1. DIJKSTRA, U . J . , W I L L A N S , J . L . , JOOSTEN, E . M . G . , GABRIEELS, F . J . M . : Ann. Neurol. 13, 325 ( 1 9 8 3 ) . - 2 . FRIGG, M . , BRUBACHER, G . : Internat. J . Vi t . Nutr. Res. 46, 3 1 4 ( 1 9 7 6 ) . 1 Neurologische Universitätsklinik, Voss-Str. 2 , D - 6 9 0 0 Heidelberg, F.R.G. 2 Department of Vitamin and Nutri t ion Research, F. Hoffmann-La Roche & Co., Basel, Switzerland. 

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Biotin levels in plasma of patients with Friedreich ataxia and other spinocerebellar degenerations

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