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 B7, H, Biotin


 .    ,      к"# $% &'(к) *+, .

   &- ./0123 45678+, . 7 '( *5*&9 1 *56: ;<% =>?5  :@A

A(+1 х
CDA<E . A   "+,FGH .  ) к)&/ &'( яJK?L)

M=(FGH . я4N @OPA G2&Q ?RA O?S M=(FGH . 456ATUV W   

45?A<% я
CDA :@A XA  . ;*YZ+, - B7, H, Biotin- R&O $[)=(\

A   "+,к)&<& !. K&' A(']R&OA O$SX)  .@]A F=& @R&OA

*5^ O?S _?5


&O O?S F=& ?RA G23' *Y`A( =&*5<E&aH ...

 bcd ,Vitamin H,Biotin ,4e.

<f 48_ bcd (Vit H - 4e).&gh . ' bcd' A& ij&OA Nbck/5%lmn3 *5@&

l  ?8_q1 ") *&9(к)&r&<E. шqg&1 t


9>m op  _ l Fu vA(, \wxy z {A( 8&65&O

9g}& "~ /04gк)&/ g0=L (&<E. <f €к) 25


CDFKK ) M=(FGH.

.&a% *<‚) : H@ƒ, /0&OA +,l... „'w3A G1 .

…к † кR7 ‡-=&lˆy (B-complex). 1916 1 4‰} кA(Šgh ‹  ( B7) 

H  < &яŒ3  = 8R R . к/ m)xLgh . Biotin 1…к tetrahydrothiophene R&Ž

кH "# W …к ureido (tetrahydroimidizalone) R&Ž *5&?& &<E. …к valeric 8_‘

*5$ _8 tetrahydrothiophene R&Ž ’к“ gN .`@Y …к .A(=&X&94+,?”&<E.

l  •x#  , R8 Gw3 ?8_q1A( , …к &яŒ3  = 8R, R8


Biotin \@Yv  _

"#5 pA( , \@Yv  l  ?8_gW-'t


l FeяŒ–' 1 "?5 "# =L (&<E.

xXg` —˜`(general view):

l ) ?Z6L , R8 \@Yv) R8 .


CD™  _
Biotin n{ mngh', \@Yv  _ l  :@K8 к)

.@=g&. ;<E :@gx8A шL ‡š v=K8 <v *5K8 =&'g›& +,'Wœ 5 š 8_‘
9кK& 1 …к "?5 "# =L (&<E. Biotin ^ :@K8 *569g 1 = 8 *+,? , gN +8_ŒžS‘

4<EŸ  =]кR=L (&<E. Biotin ?g9( €ry R8 ghl 41m ?& F=& oghS a%84/0&<E.

¡H?&, ;<E, €ry R8 9g}& F=& .Qк x¢&'g x^) R8  ?Z?”
L  1

кA4+,?”&<E.;<E €ry  < 9g}& <v Kb£&9+& x^ & '(.

xXg`&, m  4¤yR8_ шqg ’к“ €Gq .@=) к&- к)“@ biotin ?Z6L a%8+& @A

Biotin 1*& .gh'(.  g`&, .Qк <%1 9y 4'‚


CDA =&=¥ ), <]g`к) USA R8

 yzH8_, biotin A( €Gq M=(F@+& =>O&9  '(. .W$%, …к @ L ’к“ :@K8 .xXg`

:@gx8A9g A*Y+, biotin 1") .Qк& W.

Biotin ’к“ ¦L §8 =>?5& (C10 H16 O3 N2 S) . Biotin .xXg` }` кHR &r&<E. ;<E

кH "# WA Œ&+, n3‘ @¨$L) &<E. Œ&+, n3‘ @¨$L) imidazole R8 ©Eeª¦ W.

Biotin …к heterocyclic («™-) carboxylic 8_‘ S-кHR &<E. Biotin t+, &яŒ3 )

<v Œ&+, *¬gv ), . R8 pimeloyl-CoA A(&/0 ?8_g@Y?”&<E. 8-
CD™-7-

oxopelargonic 8_‘ synthase …к pyridoxal 5'-o ­® &яŒ3¯. pimeloyl-CoA, xygyƒ …к

malonyl thioester "°±A …к O@R _ghZ \@Yv  l& . 7,8-Diaminopelargonic  l& (/")

aminotransferase, NH2 <?  (AdoMet) S-adenosylmethionine *eR&O .xXg`

&<E. Dethiobiotin synthethase ATP $² 8_yG®


Q .W …к /" carbamate <v ureido R&Ž

gZ+ $%³´g`. /0к{ …к deoxyadenosyl 1 Biotin synthase reductively cleaves

AdoMet -. Dethiobiotin A Zr „' /0к{ &яŒ3¯ кHR A ;A( -=­ƒ (Fe-S)

7&'5& кA(ŠA4/0&<E ;<E =­ƒ <?, <v Oк)“\ &<E.

Cofactor :@gx8A=L §&


Biotin /0 (+) .Qк carboxylase &яŒ3 ) 1 gN +8_ŒžS‘ 4<EŸ 4^ ? …к cofactor &<E:

* µ3{-CoA carboxylase _­,

* µ3{-CoA carboxylase ¶,


* Methylcrotonyl-CoA carboxylase,
* Propionyl-CoA carboxylase,
* Pyruvate carboxylase,

4y \@Yv  l& ?8_q1, х-Š)=( .


CD™  l& :@A 9g к) *e*+, шL +,'

a%8, "#5 pA( gh8 \@Yv  l A(&/0 ?8_gWA t


l F– 1 х
CDA<E. Biotin

= eя†8 ‹ carboxylases 1 R·ˆy w3 ¸l¹ mS1-.


CD™ = t]& яº/0&94/0. ‹

biotinylation =Z&'A ATP .@=g& R8 holocarboxylase synthetase <v $%³´gк. ^

gx8A n3lк) biotin ’к“ .a[}&® "#5 p x¥¤кg`, "#5 p *g=Zg, DNA *R@RL?, R8

7ml7ˆ = ^ *5K8 .^ 8A& …к х


CA
D *5e x&76к?  *eR&9@9(P.

<к<% Biotinidase b£xy #  "#5 pAl ( biotinylate a[8 / »gh m)xLgh  \<E· biotin

=]я& FK _ я? кm=L (&<E.

Biotin 4& 1 = eя†8 4&‘ =»m&a%, 4


CDA $[HA "#5 p-w3&‘ *g=Zg …к ;<E 10-

15 кK _ …к ¼A ¥g& KD, $², tetrameric "#5 p avidin (к/ streptavidin R8

neutravidin) a_ к½RA binds. ‹ ?g9( ^ :@ x&76к .ml 7ˆA(l *eRxL gh. 2005

@gк), a_ к½RA *R¥ ?”A( biotin-streptavidin 4&‘  & .@=g& &94+,$²&<E.
Biotin ’к“ sources:

Biotin  g&1,  g @Agh =L ¨? &9(A( , .W$% \ х & Rd t_) W.

biotin к&µ&®.XEк  &a%  ) - v– 9ƒ· , /0 +,¾, K+,¾1 &/% *9P x~ A (.W$%,

+,¾ ¸v?(white) e& $² K+,¾1 &/% *9P x~ A ’к“ biotin *5G ?R±=L (&<E),

 8&, \ кg8) R8 GQgh¿A. "P? я 1  g biotin M=(F@+& 35 @gк)

70
CDFK K¯S / d (143-287 nmol / /0) Ar
l .&9 &<E.

Biotin к/ &'() A(&/0 .&'(4r1 &<E. 1940 1 '' He Sternbach R8 «n– GÀ{­

w¾ Áƒ± '' .@¨<E‚ &š *5K8 "5g&i *<g‚ & fumaric 8_‘ *eRagh .

4eG23‡Hp--

Biotin к/  H (H "]ƒ &‘ ]î", "€ry R8 9g}" F=& German *<)

=>O=(L&<E)  <  B7 . m)xLgh. Biotin ’к“ = _A


CDA :@ i ?A( A =y /Ė )к)

R8 F/0ml) 1 gvb£&O a%84/¾W. ‹ .^ 8) A(&/0, яRRA<E biotin = _A


CDA :@

i ?A(  g eR&9&$²"r < ’к“ g 4y , ?к)“@  < GQR84{ &+@9(P .

‚R&agh. xXg`&, biotin "#5 p G2ÅÆV gÇ*&1  < biocytin @&  g 1 &<E. "#5 pu

<v "#5 pAl ( OÈA Wœ *5K8 &'( Éˆ` .@=g&. ‹ *5K8 O? biotin .=(ÊË . biocytin

R8 "#5 p-G2ÅÆV biotin A(&/0 +,' „к“яÌA 1 biotin *5=L (?& ?˜`
i &;. .W$%,

a_ R&я) biotin …к 20-40% @gк) g`& = _A


CDA :@ i ?A( R&O кHR Biotin

= _A
CDA :@ i ?A( 1 =L ¨? G23X  …к x^ & @g`. ;<E  g& A(&/0 ^ biotin-

"#5 p 4&^A( …к : ’к“ x ¥Í g`& &r&<E. …к :  4&^&  g *<g‚ &

A(&/0 biotin ’к“ = _A


CDA :@ i ?A( gÎ W=(L&'Q<E  '( .
Biotin .@=) *5?& a%  .&) --

"&? biotin ¥6 "¢AÏ*YA $[H8'(, † ' "A 1 =g¡ ?к)“@ &+& biotin x¥W)

=&i@& xXg` я 1 к)“@  кA(ŠA4/0&<E., biotin xm ˜& ?к)“@ x¥W1 "0к

 nyzyF» A  < ;?g . l? g`), 4ƒ ), epileptics, mn'· @8=( GR, R8

l . g› Xg` R8 9A(4H@v+&  t?5& 1 biotin RL&9+& яRR&<E . R›`Ñ 1
.Ð[l r

4e Œ4Ò Hя& к)“@ &++  RL&9+& яRR&<E . ^> "A  4e e 

к)“@ a%8A(.

/[ª8†S

Biotin 1*& .gh'(, $%Hк", R8 iqL $² *Rˆ“R&9@9(P. ._& 1*& /0 +,¾ ("#5 p

avidin .XEк x¥W1 кHR A), Œ&+,  < .&?к&- к)“@ +к4Óy +,¾ a_ 2)... Ô

6A+  @A biotin 1*& <RM=(L&<E. /0 "#5 p 4e A( 4Ó3&‘ a%=L (&<E .

я&?”@Y) 1 biotin 1*& „'- я&?”@Y) /0 +,¾ $[l 6m¦&O  &agh. )кк) +,¾

$[l "#5 p ;@v+  @& 9g}, 7ш Aˆy&, .1m 8_, R8 +,к), к&+g к) =&4&XE&OA

*a%8к"# @+&  &agh . ‹ &/Õ5 ¯ +,¾ $[l 8& . R8 +,¾ $[l кm&a% …к Œl ÖF

"#5 p avidin . кA(ŠA4/0&<E. Avidin, GQ/0 (@&) @A denature .WA&'(A Biotin

a[к)“a['gк)&/ &r&<E .

Biotin 1*& ’к“ ˜`):

* bcWƒ Aˆy& (.1m 8_)

* к&+l кк

кTUV, к)“, ™gh, R8 яAQ&<E58 "5&?& 9(×


y …к "~ g) —/0A $²)"~ )=( @& gh*Y

Ø gÇ*&1 9g}ÉÂA((/[g}µ3– ) @A .


L , 6i5 ` , „'(·4gh R8 я'R&9& .
A> _jк{ ˜`)- ш, '(g ?”

xvк х Ø, кH …к .xXg` х \@Yv *&m`Ù,  "biotin-1*& х&" (biotin

deficient face) \&? &<E *Y`) @RÎ&agh . Biotin 1*& ’к“ @&A(? gh}?) A

@ к)L) 4¤yR8{ R8 ¡&{ ;2­˜ к) mnRRA K]`Ú? = , 4bÛA*/0A ^к @ @=¥

*Mgh A(  &agh .
R›`Ù ¦L §) biotin 1*& ’к“ к)“@ Aˆy& кHR &W. R›`Ù ¦L §) <<*Y =& biotin

?R±"# WA ¥6 *56‡&‡&a% 3-hydroxyisovaleric 8_‘ .xXg` mngh', . *RÉ^A

9>m&O&<E. g›& = 8&1 ‹ .@ш& biotin 1*&  ÜÝ@Y) '*Yryк$² @OPA

malformations(cleft palate) @9(P . GQ<E&O&<E .^ 8) $[)*Y?”W .

:@K8_ 1")

Biotin-XR? carboxylases ’к“ 1*& 9g ) кHR Gg=?v& :@K8 1") 4]ÞT

carboxylase 1*& . m)=(Lgh. ‹ &яŒ3 ) holocarboxylase synthetase  <

biotinidase 1 1"A( W. Holocarboxylase synthetase 1*& = g¥ @&?& biotin

*eR&O A(&/0 шqg& ’к“ к`) XE=L (&<E, .&'(@A 4]ÞT carboxylase *569g )

=&кgß ` 4eŒк{ R8 G23' .@ L W:. Ketolactic gкL 1  l^g}&,  &<f58

t?5 A .XEк   l  "# @Y, hyperammonemia, 9g}& ''(·ghl, <` = = ),

C?L4/0, tgÈ), .@¨<E‚ = &, .1m 8_, R8 F _.

Biotinidase 1*& =R"#  biotin g`& '(, † .<E *5K8 . &яŒ3 ) …к 1*& @gк).

Biotinidase biocytin R8 biotinyl-mnmy nà‘S (*56 holocarboxylase ’к“ proteolytic

.XÕ кg`& ?Z?”


L )) A(&/0 biotin ’к“ áHк $%³´g` R8 ?<v biotin recycles. ;<E

 g "#5 p-G2ÅÆV biotin A(&/0 biotin  L 1 к/ a_ х


CDA<E. яAg{ ˜`) ?R±&<E

кH R8 mngh' W. 9g}=&4&^ ˜`) 9g}ÉÂ, .1m 8_ (€ry Aˆy&) R8

achromotrichia (€ry 1  A*YZ+,  < @gÎ '5@ & F1Z@+&). Perosis ( к) …к к)l*L
CDA

R8 *)94/0A*Y+,) .¥*&яg& 1  &9@9(P . \@Yv  8& R8 t?5m&/

&/Õ5 ¯ (FLKS) R8  8  9g} $[3 K&^( G XE к/ =&i&9@9(P.

*e)
+8_4Ó–-- ^(
]& biotin iqL ‡· "~ &'@9(P. ;A(SH-XR? R8 -;A(SH-XR?

Œ&+, ^(
]& 1, biotin $² iqL gкL 9Œ“g 8&?5`
Cgh*g9/ R8 ?к)“@ *G=&

l Fu x¥WA( =v8& @, \ .^ 81 *G=& t


gкL t l Fu 1 ?R±&*Y 50 ?&

l Fu 8&?5` =&4&^& „'(·4gh R8 я'R&9& Œ&+,


.XE&agh . Biotin к/ t

?R±&9+&, ?g9( ^(
]& =&4&^& A> *6 GR&9+& "?5 "# xL W.

bcWƒ & gh = = )

biotin 1*& O ) R8 ˜`) M@5& 1*& ˆ8_A( 1 eyelashes R8 кA(4â 

Aˆy&, ._7 ghl , €ry Aˆy& кHR&9(A( .


Biotin &'() : a_ &'() .&'(4r1 W. oghS «$'( 7500mcg @gк)

5000mcg R&O €к) &<E. &'


CDA R8 4
CDA €ry R8  @&?
CDA ghl @¨<E‚

7r *) 21l 9>+@9(P. \ shampoos .. biotin кHR A .&'(4r1 W, †

biotin 4 Kb£&9к"# $%, GR1  *eкg


CDA *5@& &r&<E .Q ˆ8& =&<% =Z'&

&<E .

palmo plantar Pustulosis

"«"l&ƒ *Y=(L°– ) t


l Fu R8 \@Yv  _
l ) ._7 9g}&,  к R8 ;?g

.@8Gк) ’к“ .xXg` ›@ к) <RM&<E. biotin 1*& ^к *a%8к"# @+&

’к“ :@K8 derangements @OP&<E. lк{, :@K8 R8 ^к 1") . biotin

*R"A <v
CghŒž W. ‹ gÎ 8_) biotin 1*& G XE R8 < Ar@& G mL

R8 *5F*  1 к)?”&<E $[H8a%=L (W. Biotin O?Sк) …к "#5 4Ó38š agent ’к“

.A(4&^ .'A&  ’к“ O?S *5@& M@5


CD&<E. .'A&, x# R8_– 9g} , <[3b£к

 *– R©[ _=–, .mš 9g}É  < gh W‘ A  к/ ?'(*R :@K8

.xXg`?) R8 ^к *a%8к"# @+& $² biotin 1*& кHR, .&'(@A biotin O?S 

"«"l&ƒ =&'g›&1, G ^( O?S1 *5eя) .&<E&OA Xg& A&<E .

9g}É (x# 4Ò gWš 9g} É^ )

(PKU-phenylketonuria; …к .
CD™  l& ª_w
3 OÈA& a%8 к ) phenylketonuria .Q

…к .gh<[3A Gg=?v& :@K8 gh}? mlк) ?g9( 26L»' 9g}&, ;?g шqg g&1l $ g

R8 x# 4Ò ŒãWš 9g} @& 9g} *R¥ ?” кm&9( . PKU $² *5я) =&i&a% "~ g) —/0A

$²)"~ )=( @& 9g}& _ghZ) biotin *eR&9/ *¬ƒ x ¥Í =&4&XE&OA

&+@9(P. mngh?”A  g biotin ‹ =&'›1 x# 4Ò ŒãWš 9g}


Cgh 9>*4/0&<E .

ˆ*5@&-toxicity>
я&?” .^ 8) biotin .XEк x¥W1 «$'(1 g`& \, <[3 &-, *5G) Ar
l

=>O&aW. я&?”@Y) R8 _A@Y) GR "# ˆк .@=) к&- к)“@ *R _`& к†=& …к

g¾ ƒ ä «$'(1 ?ryF  gh€@YA( .&<E&9@9(P. ÜÝ@Y1l sebhorrheic 9g} <f@l

:@K8 gh}? O?S *eR&OA*YZ+, *56к *5GA( =&х GQ<E&OA =&'›1,

х &,  .XEк «$'(1 ¦vкR&9+& A(&/0 W.

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