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apts Dignan Aborpon of ale Netitinal Conte sine cea ge pane 242 Ca Cte oom Seen tai pin Donn Eas Anal Ps ep Sen tg Sempt sed eee Ni Aba Be Aca ane Costs 26D gn Pe oP het in Pi on sin Cac Din owen Ace Si ne worst ger i ‘eat dA Abby tae 26s rn Monae etry aga sae ay Cal apa east Aha isin ai Ong i a aioe ta ine ey a a pe ‘Astin iin pt dyin Mat eli oss bere ster 242 Ba Tie Din ai Ret hey 4s A Aaa amg De snes 26.1 Overview ‘Secon of digeve Mids an hgston of fod were som of he crest biochem evens oe investigate the begin ofthe eof me sense, Mor nilesions wee the discovery of byrochlre aid eecton by he trsch al czy yl fpr and th by gas uc ar saliva, spt ‘Te dcovery of gate H1 production goes back the Arsrian physician Wii Beaunont(TSS=18S3) In 1822 be Weed pain witha smack woud ‘The pain recovered rom Be wound but eineda gst ula al oping hough Be ski. Beant scized te oppo to btn and sud paste Jule fen ines ding and ae meals. Chemical analysis revealed, othe suse of hess aul blogs he presence of the sorguic aid HCL This, oe ae "eos Polypeptides generat oe ingested proteins re erated win the slimes aren by earbonypeptiases A und B. The pancreatic carborypeptiases sre Zn" mataloenzymes and poses ieee catalytic mechani than the ctboso sng peptic Te cmd aon of pancecpeptee ‘oul inthe formation of fe amin ai and sal pps of 2-S rds, Pps seat or abot 6 the amino toner a hs poirk. “Intestinal Peptidases Digest Small Peptides ‘Sine panera ie does not conan april sminopepasc ety, ial dgeston of d= and oigpepides depend on sal ites czyes. The hil ‘race of epithet ele parol chm endopepdie ad aminpeptiise acy, tao contin dpepiass Table 262). Thee prod ofthe cll "ure gestion ae fe ano seid nian inepie, wich te aborted va specific aminoacid or peptide transport sytem. rsp i nd tipi ae penal hulu whine eytoplaiecomparant blah eavethe cel. The epic ipepbas explain why praca any fee ‘sna fe fond inthe orl loo fea ea. The vit absence f peptides had previously been ken as evidence tha ami pin digestion had to proceed al the way to fee amino ais Weare absonpion could occu However, snow ealshed th lage porto feta amino nivogea is absorbed inthe iam ofsnal peptides wih subsequently. owen, and peptides containing proline and byoyprolinc or msl amin acs, oc sf slain x camosine (P-lanhisiin) or asec (falas Imi arabsorbed without ingacelulr hyo because they ar ot god subst for the insta eytopasmie peptidases P-Alanne is preset in chicken mea Free Amino Acids and Dipepties Are Absorbed by Carrer Mediated Transport ‘The mal ntestn as. ih api o abso fee amino cis ad smal pepe. Mos L-aino as can be anspor across the epium gansta cencearaton grad, although he ned fr concert espa in vv abies spe lamial coneetratons are usualy high he asa eves of (01-02 mM. Amino ae and pepe tasport inthe smallest hl the hires of arma trtapor sich x csrimiaton between D- aL sein acd and energy an tomperare dependence. fn adton, nec detect are known ecu in aman (seein Cort 263). [CLINICAL CORRELATION 263 Neutral Amino Aciduria (Hartnup Disease) anspor fnsons, ke enzymatic actions, are subject modification by mutations. An example of genes eso epi amino aid wnspt i Hany cee, arid atest fry in which the disease ety resting on the deft was rst recognize |The diseases characterize by the isi of eal nd intestinal pti cel tabs real sno ais he amen. nce ke, in which lisa ain acids each the uren of te poniral ubue though te laa, te inl a eben amino acide maniexsfeclnexrtion of ami acide inthe wie amino aid) Thien. | etc rests in malbsorption af fe aio acids om the det Thee teclincl symptoms of patents with his iscas ae mainly those duc to ceil ami ai and rismeide deen. Te elratt etre (sep 1121) at expand by = etcincy a epuopan, which ress roi For itm Tnvsiaon of pacts with Haroup case revealed th existence ist vangport systems for d= er wpspie, which ae dfn frm the ons orf amino acid, The ens eon des no af rasp of peptides, whch remains as apahway fr absorption of prcn digestion roduts Sik... Disorders of nzogen boron. In. Haris (Ed), Clini x Gasiroenterology: Familia Inherited Abnormalities, Vl 11: Londen: Saunders, 1982, pp. 47-73 ‘nthe has gnats, spt experimental expeson cloning, at Jes seve rush border speiitransport systems fore upake of aminoacid or sal peptides inthe minal membrane canbe dsinguiche: (1) fo neil amine ais with short rps hain (Set, Th, Ala) 2) fr neal ain ce with ‘coma orhydopobic sd hans (Ph, Ty Mey, Val, Lev lo (3)friminn sido Hyp; fr B-arnn ais (Al, rn) orbs rio ade faa eysie (Ly, Are Cye-Cys) (6 fra in cis (Asp, Gh) and (7) for iets (Pept) (yarn). The cacenration mechanisms or neal L-aino aid ppertobe sila to tose discus fr D-sloose ee Figure 2617). Na-dependent transport systems ive hoo ene in the minal sh onder membrane and Nx -independent ranaporters inthe cntleinl ls membrane of al esi ‘pihalalcals Sart, sor active ose arp the ney fr conceive ano sid ano derived deat fom he elsrchoricl Ne’ gat nd nly ied fom ATP. Amino sid ent chemally modified dug membrane anspor along they may be meal wii the cya opatnent ‘The bs border vansot forthe cher amino ass eetizd in ore conpliceed ways. For example, te ald amin ald transporter modes couanspar oft amino aid with 2 Na’ fons and counter tansprt wits LK on ‘Nea dipeptides are cotanspored acs the bas bed memvae with poten ants ate enerszed dough he proton lesochemial gradient crs this smemirane, However, beso of he Nit” exhnge, both rants o be sma and interepeaet. Te epi nepal seeps B-ctan shies eminent) ands pen oration fly anne ante thi cle. Fetus and Neonate Can Absorb Intact Proteins ‘The fea and neonatal sal intestines ca abr ntact proses. The upae occu by endocytosis, tha, he inemaliation of sal vesicles of plasma membrane, ‘ck contain ingested rncomoecles, Te proces slo teed pinocytosis because ral cof veils, The sl intel pinyin of pres ie ‘hgh to be importa atest of sera aso (bul) othe oping, arly in det The pioeyti pea of peas i a inp {rutin and te magaiade usually declines ae birt, Persia of ow evel of his proces beyond the sal prod may, however, be esponible for sort of sficiet quarts of maco-melecues inde antbdy frmatn. 265 Digestion and Absorption of Carbohydrates Dicand Polysaccharides Require Hydrolysis Dietary eatbotyeates provide malo potion of he diy crc equim. They consis of mono, i an poysaechards (Table 26.7). Monosaccharides eed nt hylyzed for aburton, Discharges require the saline src eyes fo ydrlyas intomoncacchardes, wile polysaccharides depend on panereatie amylase fo degradation Figure 2523) ‘Starch, jr nti plntplyeacchaside with a maleular mass of re thi 100 KD. consi of mre fier cins of gute molecule ne by 2 + glacoi ons (amos) ato branched chains with anc ots made phy «1. inkages (amps. The too 4-1 I glsocc onde ‘shout 20: Glycogen an simalpolysacchare simian sruczeto amylopectin The two srpomds fern ems ofthe imber of branch pois wish ‘ccurmare fag nscogn ytd starch and pycogen are tacked bythe endowaechaidase e-amylase present in sv and parti jie (Figue 2624, ydaion ofthe opsacsaries occurs rng sing assent fr ficient digestion, Amyls s specifier eral a1 +-ghcosie bond 1,6 bonds are not tacked, or are a1 bonds eos us tha serve as branch pons. The pares snerzyne is secreted in ng exces reatve to sar ike an Digestion adeno a byte [CLINICAL CORRELATION 264 |Disaccharidase Deficiency resinal debaters ae entry spots hams | Deticeey can be preset in oe enzyme or several enzymes ora vaely of reasons | genet deer, physolgildalne wit age, othe esl of injures othe mace), ord isccharidses, cts ete most ommon ems wih an abso olive erin. which i exprcned as milk wolcance. The consequences ofan nab to lye lca the upper nl seine ae abi to sbear noe a bacral mentation of gested lee inthe lower sl sestne. Bacterial ementiaion esis in th rodtin of essen of gat an uence) and erotically actives ia daw water ino the seins famen ices). The ace a yop has rey bee pally colyzed ring the eentin proces of making yop. Ths ndvidals wih ats deficie can ofl tolerate yogurts thanunfemented diy prs, [The exyme lactase is commer avaiable preteat miko that te laceses [Bul HA. and Grant RG. Lactose intolerance. An. Rev Med. 41:41, 1990. ‘smoreinprtn than the salivary erayne ram gestive pont of view. The prods of he digestion by a-amylase are sin the dsaceharie male, the trisaccharide maltotiose, and so-called a-litdertrins coining on average ight glucose units wi be or mare a1. lucscic bon. Fin hydrops of and lgosechardes to monosschrids is ard out by surface enzymes of the sal inset el (Tale 268), Mostof th ‘rice oligosaccharides ate exoenzymes tha clave off ne monosaccharide wa ime rem the nonredcing er The cacy he a-glncoidate ional ‘uk grater than ha noo for completion ofthe digestion of tech Salty, her aly exces cpa for sucone (able saga) yds o dicary nuke In contrast, B-glactosidase lactase) canbe tenting in humans fr hylan ulation of aos, the major mak abo Ge Cin. Con 264), oligo and plyscerid tht not hyyz by asm nor inten sue enzymes canot he rb ther they rch the lower ra of ‘hens, Wich rom the lower eum an miss hata. Bacteria can lize many of the ema arbobyes boca they pos any mares of ‘echidass th humars Monosaccharides that rereleased as a est of bacterial enzymes ae redoiary metalized arabica by the baceia ‘hemeles,esulng in degadsion products Soh as sharia fat acd, tate, byrogen gs (2H), mca (CH), ad carbon 4 4.) oxide (00), These compounds cn case uid secretion, nee intestinal ott, al cramps eer because of increas nasal sms Presa and Adsension ofthe pt 8 re iat fof ti tater deradaton prods onthe ntl meas, ‘The well-nown problem of ulnar ingestion of leguminous seeds (bans, peas, and soja) is caused by oligosaccharides, which canot behysolyad by man neil enzymes, The ogni ceca mode cos 0 wich one or mote alco oii ar nko. The horde bond of aan ae ‘he confguation, which eto spt baci enzyns. The inlet Siar ois fay raflnose (see Table 26.) ‘real dsccaride al occa your mushrooms egies special sesh, rel [Monosaccharides Are Absorbed by Carrer-Medited Transport ‘Toe msjr monosaccharides tat esl rom distin of - and polysaccharide ae Dose, Daan, nd D-iutose. Absorption of these nd oter minor monosaccharides ae care-madated process that exhibit sch features sSbstate specifiy, stereospecific, stan kinetics, and ton by specie ‘shite. Atleast two types of monosaccharide anspor catalyze monosacharde uptake fom the me nto he cel (1) Na-monosaceharide cotransporter, exiting probably a etre of 5-KDs pep, ha igh spec for D-glerse ant D-placove sd easiest" sr aorten (SGT) a (2) Na Independent, facitated diffusion ipo of monnachide sposystn wih pei for D- tse (GI-UTS). In aio, 2 Na-ndependent ‘monosaccharide transporter (GLUTZ), casing of 57-ADa pepe), which aco alee mnosachardes,srset ia the conan plasna meme. (GLUT ie abo acted inte iver and Kae. and othe members Fhe GLUT fam of cose anspertss ae found in allel All GLUT auspones medias nssapled D-glucose ox do its concentration waders. GLUT of gt, ver, and kidney moves D-ghsoas ou of theo no the aod under physiological conditions, win oer tssus GLUTL (nett and brain) othe insl-sestive GLUT (a ita muscle tse) re miny inven Degas uptake. Propeiesof nesta SGLT! and of GLUT2 are compared in Table 268, ad theron transepithelial loose absarpton stated in Fig 2618. eget igure 2625 Geeta Alea edict pases canbe singed Fgue 2627: () hyo of ways to ee fy aids and monanesllyerls (2) solubilization by ‘detegenss (ile acids) and wenspor fm he ies! lumen toward te cel sre (3) pte of fe fay ais and mononylyeol note cel and sys se pat ad em li ale yen Sy ym en sete Lumen Enoroeyte Lymph apitiary Featy aid iar 2627 Dsin nd tsrs ofi ke? ‘Changs phy sat arial gen tevin Te BG, dey ceo FA, ay Lipids Ave Digested by Gastric and Pancreatic Lipases Digestion of ips tt in the stacy av al-stable pase, os of whi hough oot om gaa the hack ofthe ong, However, the ata yal islow beemse te sgestd wiacyeuos oem a separa pd pase witha ited water~ipi inrface- The pss abs oat inefae and envers ‘iacygiyeeos ino ay ais and icsgyeeras Figure 2628). The inperanceo he nia ysis at some of he waer-imniscble wiacylyerols are converted to products that possess beth polar and nonpolar groups. Such suracive products spontaneously adsorb to wate meraces ad oon hyophiic surace pid dope thereby providing sabe inerfice wit the agicous exvreamest. At constant volume of he pd pase any increase in inertial are rouees dispersion of tepid phase int salle droplets emulsicabon) and proves more sites fo asorpion of ore hase males ‘The mse ene fo ily yey the panereate pase (gue 25.29) Thi ere i specif forests in he a-poton of esl and pes lang fat acd with ve dane eatbo tans. Hyclas y he pancreatic enzyee aw acc a Te water-ipid itsereof enon dope. The prodbets ar re fatty acids and B-mononeylglyeerols. The pure frm ofthe zy sony hibited by he ile acd tha normally ae preset ihe sal ‘none ding pi ipso, The role of non it vercomey clips al prot (12 KDs) thal bn both the water-pineae sd ips, ‘herby anchoring and activating Ieee. is sacred y the pares procs an depends on ni removal oe NA trial decapie fr ll sci Pancreatic uc aso contains note les peste iid esterase, whic cs on cholesterol estes, monoglyeeies,o ote iid esters, suchas esters of vitzmin A sv cabo aid In coast ayer pas, his pid exer requis bile eis or activ, Phospholipids are hay by pei phospholpass. Pane secetions re especialy rich nthe proenzye for phospholipase A, (Figue 26:30), As other rete proctzymes,hisone ils acivated by tpn Phospelpse A, rues i cis for acy. Bile Aeid Micelles Solublice Lips during Digestion Bile acide ilo detepent thst ae syatheszed by he vera sere conjugates of lyelne or taurine withthe il int the duodenum At physi vals, thy are presets anions, whi fave deerget igure 2630 Mocs of soe phorbol A, opens, Theretie the tsi ald and bile salts ar of use nechangebly (Figue 26.31), Bile ads at pH values above the pK (Table 26.10) ‘eves Zor agregts al conczatns above 2-5 aM, Thos aggregates a aed nelle, andthe minimal concearsin nocesary fx mise emai ‘he era mielar concentration gue 262. Taebie ais in icles arin quia with hse Boe in sluion. Ths mses i conas emus lipids, reali stucres wt well-defined sist are much salle than emus dope. Micele sie ply range beween 40 and 600m depending onbil ned eanenati nd ten of tl acd ii ‘The arangemens of ie aids in mies such tut the hyophobic orons ae removed fom contac wit water, while hyopilis groups remn expos the sre. The hysrophobi region of ic ass armed by one sre of th feed rng syst, whe the carbonate rslaton andthe hyroxy rou onthe esd ofthe ng syster are hope Sines th major ding fos fr miele formation are th ema of aol, hyeophabc groups from andthe ‘seracon of polar group with water moles, be tabu of plarand apolar eons pce some corso he trochaniclamangement oie ad moles witina mice. Furi sed mlccls ar suicietofrm a ey simple micelle as shown in Figure 2633, Biles mele an solbiie ahr pds, sch s phospholipids nd fit cds. These mixed micelles ve dsl shapes, whee te phospholipids nd ty acds fanmaiayer andthe ile cis ‘occupy the edge postions, rendering the edge of te disk hyopbilc (Figure 26.8), Within the mixed postop acs mses, ter wae nso pid, suck as cholesterol, canbe eeommodated api thereby "slbiized™ (fr potesal problems se Cl Cr. 26.5), sonar = & Diagramme repeenttn aha ct me appro Sa DML ‘ictim Bop e817 ag 180 Septet porn ene a gy SE | Piure 2036 seta in sand Wer pr eer me it cones oy aes yey espn tds potted wie mer "zw ed pe Carey M Cin AM ‘Av Popper Sec rl (Se) Phe Ee Bile nd "Mag Sew Vo Raven a9 During iayeyeo digestion, fest acids and mancocyllycerl ae released athe stface of ft mlson droplets. Tn const 9 iaygyoeos, whic re water soluble ay acs nd monoacyyeol ae igh wat-slbl, apd molecules tthe sure equate wh hose soon, Ths latin tur become ‘neuporated into bile acd cele Ths the rau of acylyeol ysis continuous) transered fam emulsion ope othe mice (ee Figure 2627). Miceli provi the major vehi for moving pie ho teins men tthe cll aae where ssurption cus Besa the hi layer net othe sl sii ocely mixed, tbe sor spt estas for soe Kans, P.Apopoprosin B:sructral and metabolic heterogesiy. An. Rev | Physiol 48:67, 1983; and Kane, JP and Havel JI: C.K Sesve, AL Beaudet, W.. Si, and D. Valle (Eds), The Metabolic and Molecular Bases of |ahered Disease, Vol, The New York: MeGraw-til, 1995, p. 1853, ‘tu cos this" usied" fi lyri diison down he concearaton gic With his type of tansport mechanism, te delivery a of nae athe cel suace 's poporiona te once dirence between funinal ul phase ap cel sure. Obouly, be nse tid ayer rescas robles fr saingly soluble lorie ston, nth esol delivery rts carmat be achived, hl aed micelles overcome hs roblen far ips by mere her ef concern inte unset lye The nese in wasp rate eat proportion othe mene in lective concerto ad a be 100-fold ove ht oid soluble fay acd accordance wth he ent soli fy aks clo a iid adele This eases hx and effcve oncentatos olds because the isin const, another parame tha denies the a, oly shy smal ote mixed cls a compared oid ‘molec fein slun, Ths fica iid bsorpton depends oa the presen of iis bs aso oui” ingested bald pis ia cele. thease file asthe absorption of ticyg}eols dos no completely stp, although the een i esta redo. Theresa absorption depends on the Sight water sob ofthe fe faty aes and monseylyerols, Unasorbed lipids reach he lower ese where a sal ar canbe metabolized by bata. The bulk of urabsorbs pis however is exerted wit he stool his scaled eatorrhea). “Mics also transport choestol andthe ipi-soible vitamins AD, Hand K ough te unsied Hid layers. Bai sero sable ese fr te sbsrpon Most sorbed Lipids Are Incorporated into Chylomicrons Uae ftps bythe ciel eis occu hy dision hough th plasma membrane. Absorption sity complet fr fat acids and monoayglyceols, which axes wee-slble isles fc! water-inslble ps, For example, uly 30-40% ofthe tary eolesteol is absorbed. ‘Within eines eels, heft of bre ay aids depends on chain length, Fatyacds of medlum cain length (6-10 carbon atoms) pas hough te cll ‘0 te poral ood witour madison Lang-chain fy acids >13 carbon atoms) become Bou oa oso, speccalyInestinal fatty acid-binding poten [FABP) and are tarspoted tothe endoplasmic, where they re estes into tnacyglyeols. lycra for this ree s derive rom the absrbed 2-mnoseyipyorl a, 3 mor dere om poss The esas ayes or ip lbles to whih surest phospholipids nd spa roti, red apolipoproteins, ak The poics migrate win merino wesc tg the Gol the basolateral plata membrane. They ‘eile othe erly space by fas of he vesls wh he Basta plasma memane. Because the pd plabule an be several mice ‘dam and because they lave the tse via yap vessel ey a eae ehylomlerons (y= mith tha spss in he isin anh vss, Ince and he rai dst ale pid ea: ches derived fom the Greck cpl, which meas jue) The mesial popopotis ae diet ern Sm those ofthe iver and are designated AI and B, Apolipoprotin Bis eral for chylomicron reese fom entero (se Cin, Co 268). ‘Whe try motu-

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