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CARBOHYDRATES

Disturbance in Human Metabolism

Abdul Azeem
Roll # 20 MS Food Science and Technology Session: 2012-2014

Contents
Contents.......................................................................................................................................................2 INTRODUCTION....................................................................................................................................... TY!ES......................................................................................................................................................... CARBOHYDRATE METABO"ISM......................................................................................................... IM!ORTANCE........................................................................................................................................... METABO"IC !ATH#AYS.......................................................................................................................$ %"UCORE%U"ATION..............................................................................................................................$ HUMAN DISEASES O& CARBOHYDRATE METABO"ISM................................................................' Diabetes mellitus.....................................................................................................................................' %alactosemia...........................................................................................................................................( Here)itar* +ructose intolerance................................................................................................................, %l*co-en stora-e )isease........................................................................................................................., RE&ERENCES............................................................................................................................................/

INTRODUCTION
A carbo0*)rate is a lar-e biolo-ical molecule1 or macromolecule1 consistin- onl* o+ carbon 2C31 0*)ro-en 2H31 an) o4*-en 2O31 usuall* 5it0 a 0*)ro-en.o4*-en atom ratio o+ 2.6 2as in 5ater37 in ot0er 5or)s1 5it0 t0e em8irical +ormula Cm2H2O3n 250ere m coul) be )i++erent +rom n3. Some e4ce8tions e4ist7 +or e4am8le1 )eo4*ribose1 a su-ar com8onent o+ DNA1 0as t0e em8irical +ormula C'H69O$. Carbo0*)rates are tec0nicall* 0*)rates o+ carbon7 structurall* it is more accurate to :ie5 t0em as 8ol*0*)ro4* al)e0*)es an) ;etones. All carbo0*)rates s0are a -eneral +ormula o+ a88ro4imatel* CnH2nOn7 -lucose is C (H62O(. Monosacc0ari)es ma* be c0emicall* bon)e) to-et0er to +orm )isacc0ari)es suc0 as sucrose an) lon-er 8ol*sacc0ari)es suc0 as starc0 an) cellulose.

TYPES
T0e carbo0*)rates 2sacc0ari)es3 are )i:i)e) into +our c0emical -rou8in-s. monosacc0ari)es1 )isacc0ari)es1 oli-osacc0ari)es1 an) 8ol*sacc0ari)es. In -eneral1 t0e monosacc0ari)es an) )isacc0ari)es1 50ic0 are smaller 2lo5er molecular 5ei-0t3 carbo0*)rates1 are commonl* re+erre) to as su-ars. T0e 5or) sacc0ari)e comes +rom t0e %ree; 5or) <=>?@ABC 2sD;;0aron31 meanin- Esu-ar.E #0ile t0e scienti+ic nomenclature o+ carbo0*)rates is com8le41 t0e names o+ t0e monosacc0ari)es an) )isacc0ari)es :er* o+ten en) in t0e su++i4 Fose. &or e4am8le1 -ra8e su-ar is t0e monosacc0ari)e -lucose1 cane su-ar is t0e )isacc0ari)e sucrose1 an) mil; su-ar is t0e )isacc0ari)e lactose.

CARBOHYDRATE METABOLISM
Denotes t0e :arious bioc0emical 8rocesses res8onsible +or t0e +ormation1 brea;)o5n an) intercon:ersion o+ carbo0*)rates in li:in- or-anisms. T0e most im8ortant carbo0*)rate is -lucose1 a sim8le su-ar 2monosacc0ari)e3 t0at is metaboliGe) b* nearl* all ;no5n or-anisms. %lucose an) ot0er carbo0*)rates are 8art o+ a 5i)e :ariet* o+ metabolic 8at05a*s across s8ecies. 8lants s*nt0esiGe carbo0*)rates +rom carbon )io4i)e an) 5ater b* 80otos*nt0esis storin- t0e absorbe) ener-* internall*1 o+ten in t0e +orm o+ starc0 or li8i)s. !lant com8onents are consume) b* animals an) +un-i1 an) use) as +uel +or cellular res8iration. O4i)ation o+ one -ram o+ carbo0*)rate *iel)s a88ro4imatel* $ ;cal o+ ener-* an) +rom li8i)s about H ;cal. Ener-* obtaine) +rom metabolism 2e.-. o4i)ation o+ -lucose3 is usuall* store) tem8oraril* 5it0in cells in t0e +orm o+ AT!. Or-anisms ca8able o+ aerobic res8iration metaboliGe -lucose an) o4*-en to release ener-* 5it0 carbon )io4i)e an) 5ater as b*8ro)ucts.

IMPORTANCE
Carbo0*)rates are a su8erior s0ortFterm +uel +or or-anisms because t0e* are sim8ler to metaboliGe t0an +ats or t0ose amino aci)s 2com8onents o+ 8roteins3 t0at can be use) +or +uel. In animals1 t0e most im8ortant carbo0*)rate is -lucose. T0e concentration o+ -lucose in t0e bloo) is use) as t0e main control

+or t0e central metabolic 0ormone1 insulin. Starc01 an) cellulose in a +e5 or-anisms 2e.-.1 some animals 2suc0 as termites3 an) some microor-anisms 2suc0 as 8rotists an) bacteria331 bot0 bein- -lucose 8ol*mers1 are )isassemble) )urin- )i-estion an) absorbe) as -lucose. Some sim8le carbo0*)rates 0a:e t0eir o5n enG*matic o4i)ation 8at05a*s1 as )o onl* a +e5 o+ t0e more com8le4 carbo0*)rates. T0e )isacc0ari)e lactose1 +or instance1 reIuires t0e enG*me lactase to be bro;en into its monosacc0ari)es com8onents7 man* animals lac; t0is enG*me in a)ult0oo). Carbo0*)rates are t*8icall* store) as lon- 8ol*mers o+ -lucose molecules 5it0 -l*cosi)ic bon)s +or structural su88ort 2e.-. c0itin1 cellulose3 or +or ener-* stora-e 2e.-. -l*co-en1 starc03. Ho5e:er1 t0e strona++init* o+ most carbo0*)rates +or 5ater ma;es stora-e o+ lar-e Iuantities o+ carbo0*)rates ine++icient )ue to t0e lar-e molecular 5ei-0t o+ t0e sol:ate) 5aterFcarbo0*)rate com8le4. In most or-anisms1 e4cess carbo0*)rates are re-ularl* catabolise) to +orm acet*lFCoA1 50ic0 is a +ee) stoc; +or t0e +att* aci) s*nt0esis 8at05a*7 +att* aci)s1 tri-l*ceri)es1 an) ot0er li8i)s are commonl* use) +or lon-Fterm ener-* stora-e. T0e 0*)ro80obic c0aracter o+ li8i)s ma;es t0em a muc0 more com8act +orm o+ ener-* stora-e t0an 0*)ro80ilic carbo0*)rates. Ho5e:er1 animals1 inclu)in- 0umans1 lac; t0e necessar* enG*matic mac0iner* an) so )o not s*nt0esiGe -lucose +rom li8i)s1 t0ou-0 -l*cerol can be con:erte) to -lucose. J

METABOLIC PATHWAYS
Carbon fixation1 or 80otos*nt0esis1 in 50ic0 CO2 is re)uce) to carbo0*)rate. Glycolysis F t0e o4i)ation metabolism o+ -lucose molecules to obtain AT! an) 8*ru:ate. !*ru:ate +rom -l*col*sis enters t0e Krebs c*cle1 also ;no5n as t0e citric aci) c*cle1 in aerobic or-anisms a+ter mo:in- t0rou-0 8*ru:ate )e0*)ro-enase com8le4. The pentose phosphate pathway1 50ic0 acts in t0e con:ersion o+ 0e4oses into 8entoses an) in NAD!H re-eneration. NAD!H is an essential antio4i)ant in cells 50ic0 8re:ents o4i)ati:e )ama-e an) acts as 8recursor +or 8ro)uction o+ man* biomolecules. Glycogenesis F t0e con:ersion o+ e4cess -lucose into -l*co-en as a cellular stora-e mec0anism7 t0is 8re:ents e4cessi:e osmotic 8ressure buil)u8 insi)e t0e cell
Glycogenolysis F t0e brea;)o5n o+ -l*co-en into -lucose1 50ic0 8ro:i)es a -lucose su88l* +or -lucoseF )e8en)ent tissues.

Gluconeogenesis F de novo s*nt0esis o+ -lucose molecules +rom sim8le or-anic com8oun)s. an e4am8le in 0umans is t0e con:ersion o+ a +e5 amino aci)s in cellular 8rotein to -lucose.
Metabolic use o+ -lucose is 0i-0l* im8ortant as an ener-* source +or muscle cells an) in t0e brain1 an) re) bloo) cells.

GLUCOREGULATION
%lucore-ulation is t0e maintenance o+ stea)* le:els o+ -lucose in t0e bo)*7 it is 8art o+ 0omeostasis1 an) so ;ee8s a constant internal en:ironment aroun) cells in t0e bo)*.

T0e 0ormone insulin is t0e 8rimar* re-ulator* si-nal in animals1 su--estin- t0at t0e basic mec0anism is :er* ol) an) :er* central to animal li+e. #0en 8resent1 it causes man* tissue cells to ta;e u8 -lucose +rom t0e circulation1 causes some cells to store -lucose internall* in t0e +orm o+ -l*co-en1 causes some cells to ta;e in an) 0ol) li8i)s1 an) in man* cases controls cellular electrol*te balances an) amino aci) u8ta;e as 5ell. Its absence turns o++ -lucose u8ta;e into cells1 re:erses electrol*te a)Lustments1 be-ins -l*co-en brea;)o5n an) -lucose release into t0e circulation b* some cells1 be-ins li8i) release from li8i) stora-e cells1 etc. T0e le:el o+ circulator* -lucose 2;no5n in+ormall* as Ebloo) su-arE3 is t0e most im8ortant si-nal to t0e insulinF8ro)ucin- cells. Because t0e le:el o+ circulator* -lucose is lar-el* )etermine) b* t0e inta;e o+ )ietar* carbo0*)rates1 )iet controls maLor as8ects o+ metabolism :ia insulin. In 0umans1 insulin is ma)e b* beta cells in t0e 8ancreas1 +at is store) in a)i8ose tissue cells1 an) -l*co-en is bot0 store) an) release) as nee)e) b* li:er cells. Re-ar)less o+ insulin le:els1 no -lucose is release) to t0e bloo) +rom internal -l*co-en stores +rom muscle cells. T0e 0ormone -luca-on1 on t0e ot0er 0an)1 0as an e++ect o88osite to t0at o+ insulin1 +orcin- t0e con:ersion o+ -l*co-en in li:er cells to -lucose1 50ic0 is t0en release) into t0e bloo). Muscle cells1 0o5e:er1 lac; t0e abilit* to e48ort -lucose into t0e bloo). T0e release o+ -luca-on is 8reci8itate) b* lo5 le:els o+ bloo) -lucose. Ot0er 0ormones1 notabl* -ro5t0 0ormone1 cortisol1 an) certain catec0olamines 2suc0 as e8ine80erine3 0a:e -lucore-ulator* actions similar to -luca-on.

HUMAN DISEASES OF CARBOHYDRATE METABOLISM

Diabetes mellitus
Sim8l* diabetes1 is a -rou8 o+ metabolic )iseases in 50ic0 a 8erson 0as 0i-0 bloo) su-ar1 eit0er because t0e 8ancreas )oes not 8ro)uce enou-0 insulin1 or because cells )o not res8on) to t0e insulin t0at is 8ro)uce).J2M T0is 0i-0 bloo) su-ar 8ro)uces t0e classical s*m8toms o+ 8ol*uria 2+reIuent urination31 8ol*)i8sia 2increase) t0irst31 an) 8ol*80a-ia 2increase) 0un-er3. T0ere are t0ree main t*8es o+ )iabetes mellitus 2DM3.

T*8e 6 DM results +rom t0e bo)*Ns +ailure to 8ro)uce insulin1 an) currentl* reIuires t0e 8erson to inLect insulin or 5ear an insulin 8um8. T0is +orm 5as 8re:iousl* re+erre) to as EinsulinF)e8en)ent )iabetes mellitusE 2IDDM3 or ELu:enile )iabetesE.

T*8e 2 DM results +rom insulin resistance1 a con)ition in 50ic0 cells +ail to use insulin 8ro8erl*1 sometimes combine) 5it0 an absolute insulin )e+icienc*. T0is +orm 5as 8re:iousl* re+erre) to as non insulinF)e8en)ent )iabetes mellitus 2NIDDM3 or Ea)ultF onset )iabetesE.
T0e t0ir) main +orm1 -estational )iabetes1 occurs 50en 8re-nant 5omen 5it0out a 8re:ious )ia-nosis o+ )iabetes )e:elo8 a 0i-0 bloo) -lucose le:el. It ma* 8rece)e )e:elo8ment o+ t*8e 2 DM.

Ot0er +orms o+ )iabetes mellitus inclu)e con-enital )iabetes1 50ic0 is )ue to -enetic )e+ects o+ insulin secretion1 c*stic +ibrosisFrelate) )iabetes1 steroi) )iabetes in)uce) b* 0i-0 )oses o+ -lucocorticoi)s1 an) se:eral +orms o+ mono-enic )iabetes. Untreate)1 )iabetes can cause man* com8lications. Acute com8lications inclu)e )iabetic ;etoaci)osis an) non;etotic 0*8erosmolar coma. Serious lon-Fterm com8lications inclu)e car)io:ascular )isease1 c0ronic renal +ailure1 an) )iabetic retino8at0* 2retinal )ama-e3. A)eIuate treatment o+ )iabetes is t0us im8ortant1 as 5ell as bloo) 8ressure control an) li+est*le +actors suc0 as sto88in- smo;in- an) maintainin- a 0ealt0* bo)* 5ei-0t.
All +orms o+ )iabetes 0a:e been treatable since insulin became a:ailable in 6H261 an) t*8e 2 )iabetes ma* be controlle) 5it0 me)ications. Insulin an) some oral me)ications can cause 0*8o-l*cemia 2lo5 bloo) su-ars31 50ic0 can be )an-erous i+ se:ere. Bot0 t*8es 6 an) 2 are c0ronic con)itions t0at cannot be cure). !ancreas trans8lants 0a:e been trie) 5it0 limite) success in t*8e 6 DM7 -astric b*8ass sur-er* 0as been success+ul in man* 5it0 morbi) obesit* an) t*8e 2 DM. %estational )iabetes usuall* resol:es a+ter )eli:er*. Diabetes mellitus is a c0ronic )isease1 +or 50ic0 t0ere is no ;no5n cure e4ce8t in :er* s8eci+ic situations. Mana-ement concentrates on ;ee8in- bloo) su-ar le:els as close to normal 2Eeu-l*cemiaE3 as 8ossible1 5it0out causin- 0*8o-l*cemia. T0is can usuall* be accom8lis0e) 5it0 )iet1 e4ercise1 an) use o+ a88ro8riate me)ications 2insulin in t0e case o+ t*8e 6 )iabetes7 oral me)ications1 as 5ell as 8ossibl* insulin1 in t*8e 2 )iabetes3. !atient e)ucation1 un)erstan)in-1 an) 8artici8ation is :ital1 since t0e com8lications o+ )iabetes are +ar less common an) less se:ere in 8eo8le 50o 0a:e 5ellFmana-e) bloo) su-ar le:els. T0e -oal o+ treatment is an HbA6C le:el o+ (.'O1 but s0oul) not be lo5er t0an t0at1 an) ma* be set 0i-0er. Attention is also 8ai) to ot0er 0ealt0 8roblems t0at ma* accelerate t0e )eleterious e++ects o+ )iabetes. T0ese inclu)e smo;in-1 ele:ate) c0olesterol le:els1 obesit*1 0i-0 bloo) 8ressure1 an) lac; o+ re-ular e4ercise. S8ecialise) +oot5ear is 5i)el* use) to re)uce t0e ris; o+ ulceration1 or reFulceration1 in atFris; )iabetic +eet.

Galactosemia
2Britis0 Galactosaemia3 is a rare -enetic metabolic )isor)er t0at a++ects an in)i:i)ualNs abilit* to metaboliGe t0e su-ar -alactose 8ro8erl*. Alt0ou-0 t0e su-ar1 lactose1 metaboliGes to -alactose1 -alactosemia is not relate) to an) s0oul) not be con+use) 5it0 lactose intolerance. %alactosemia +ollo5s an autosomal recessi:e mo)e o+ in0eritance t0at con+ers a )e+icienc* in an enG*me res8onsible +or a)eIuate -alactose )e-ra)ation.
&rie)ric0 %o88ert 26/,9P6H2,31 a %erman !0*sician1 +irst )escribe) t0e )isease in 6H6,1 5it0 its cause as a )e+ect in -alactose metabolism bein- i)enti+ie) b* a -rou8 le) b* Herman Kalc;ar in 6H'(. Its inci)ence is about 6 8er (91999 birt0s +or 8eo8le o+ Euro8ean ancestr*. In ot0er 8o8ulations t0e inci)ence rate )i++ers. %alactosemia is ten times more common 5it0in t0e Iris0 Tra:eller 8o8ulation.

"actose in +oo) 2suc0 as )air* 8ro)ucts3 is bro;en )o5n b* t0e enG*me lactase into -lucose an) -alactose. In in)i:i)uals 5it0 -alactosemia1 t0e enG*mes nee)e) +or +urt0er metabolism o+ -alactose are se:erel* )iminis0e) or missin- entirel*1 lea)in- to to4ic le:els o+ -alactose 6F 80os80ate in :arious tissues as in t0e case o+ classic -alactosemia1 resultin- in 0e8atome-al* 2an enlar-e) li:er31 cirr0osis1 renal +ailure1 cataracts1 brain )ama-e1 an) o:arian +ailure. #it0out treatment1 mortalit* in in+ants 5it0 -alactosemia is about ,'O.
%alactosemia is in0erite) in an autosomal recessi:e manner1 meanin- a c0il) must in0erit one )e+ecti:e -ene +rom eac0 8arent to s0o5 t0e )isease. HeteroG*-otes are carriers1 because t0e* in0erit one normal -ene an) one )e+ecti:e -ene. Carriers s0o5 no s*m8toms o+ -alactosemia.

Hereditary fructose intolerance


It is an inborn error o+ +ructose metabolism cause) b* a )e+icienc* o+ t0e enG*me al)olase B. In)i:i)uals a++ecte) 5it0 H&I are as*m8tomatic until t0e* in-est +ructose1 sucrose1 or sorbitol. I+ +ructose is in-este)1 t0e enG*matic bloc; at al)olase B causes an accumulation o+ +ructoseF6F 80os80ate. T0is accumulation 0as )o5nstream e++ects on -luconeo-enesis an) re-eneration o+ a)enosine tri80os80ate 2AT!3. S*m8toms o+ H&I inclu)e :omitin-1 0*8o-l*cemia1 Laun)ice1 0emorr0a-e1 0e8atome-al*1 0*8eruricemia an) 8otentiall* ;i)ne* +ailure. #0ile H&I is not clinicall* a )e:astatin- con)ition1 t0ere are re8orte) )eat0s in in+ants an) c0il)ren as a result o+ t0e metabolic conseIuences o+ H&I. Deat0 in H&I is al5a*s associate) 5it0 8roblems in )ia-nosis.
H&I is an autosomal recessi:e con)ition cause) b* mutations in t0e ALDOB -ene1 locate) at HI22. . Dia-nosis o+ H&I is t*8icall* sus8ecte) base) on )ietar* 0istor*1 es8eciall* in in+ants 50o become s*m8tomatic a+ter breast +ee)in- is su88lemente) b* +ructose containin- +oo)s. T0is sus8icion is t*8icall* con+irme) b* molecular anal*sis. Treatment o+ H&I is base) aroun) strict a:oi)ance o+ +ructose in t0e )iet. Ol)er 8atients 5it0 H&I t*8icall* sel+Fselect a )iet lo5 in +ructose1 e:en be+ore bein- )ia-nose).

Glycogen storage disease


2GSD1 also glycogenosis an) dextrinosis3 is t0e result o+ )e+ects in t0e 8rocessin- o+ -l*co-en s*nt0esis or brea;)o5n 5it0in muscles1 li:er1 an) ot0er cell t*8es. %SD 0as t5o classes o+ cause. -enetic an) acIuire). %enetic %SD is cause) b* an* inborn error o+ metabolism 2-eneticall* )e+ecti:e enG*mes3 in:ol:e) in t0ese 8rocesses. In li:estoc;1 acIuire) %SD is cause) b* into4ication 5it0 t0e al;aloi) castanos8ermine.

O:erall1 accor)in- to a stu)* in Britis0 Columbia1 a88ro4imatel* 2. c0il)ren 8er 699 999 birt0s 26 in $ 19993 0a:e some +orm o+ -l*co-en stora-e )isease. In t0e Unite) States1 t0e* are estimate) to occur in 6 8er 291999F2'1999 birt0s. A Dutc0 stu)* estimate) it to be 6 in $91999.

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