Stem_VV0 Cells_NN2 from_II Reprogrammed_JJ Adult_NN1 Cells_NN2 Found_VVD to_TO Bring_VV0 Along_RP Genetic_JJ Defects_NN2 of_IO Their_APPGE Donors_NN2 Although_CS liver_NN1 cells_NN2 created_VVN from_II induced_JJ pluripotent_JJ stem_NN1 cells_NN2 reflect_VV0 the_AT defects_NN2 of_IO their_APPGE source_NN1 ,_, they_PPHS2 may_VM offer_VVI a_AT1 new_JJ tool_NN1 to_TO study_VVI inherited_JJ disorders_NN2 ._. 
Realistic_JJ stem_NN1 cell_NN1 therapies_NN2 to_TO replace_VVI diseased_JJ or_CC damaged_JJ tissue_NN1 may_VM still_RR be_VBI years_NNT2 away_RL ,_, but_CCB researchers_NN2 have_VH0 uncovered_VVN a_AT1 promising_JJ new_JJ use_NN1 for_IF these_DD2 undifferentiated_JJ cells_NN2 :_: they_PPHS2 can_VM be_VBI programmed_VVN to_TO become_VVI patient-specific_JJ laboratory_NN1 models_NN2 of_IO inherited_JJ liver_NN1 disease_NN1 ._. 
These_DD2 new_JJ tools_NN2 could_VM be_VBI useful_JJ for_IF teasing_VVG out_RP disease_NN1 mechanisms_NN2 and_CC testing_VVG new_JJ drug_NN1 therapies_NN2 ._. 
Scientists_NN2 from_II the_AT University_NN1 of_IO Cambridge_NP1 's_GE Institute_NN1 for_IF Medical_JJ Research_NN1 obtained_VVD skin_NN1 cells_NN2 from_II 10_MC patients_NN2 --_NN1 seven_MC who_PNQS had_VHD various_JJ forms_NN2 of_IO inherited_JJ liver_NN1 disease_NN1 ,_, and_CC three_MC healthy_JJ controls_NN2 ._. 
They_PPHS2 reprogrammed_VVD the_AT skin_NN1 cells_NN2 ,_, rejuvenating_VVG them_PPHO2 into_II an_AT1 embryolike_JJ state_NN1 (_( using_VVG the_AT four-gene_JJ approach_NN1 described_VVN in_II 2007_MC )_) ._. 
The_AT researchers_NN2 then_RT cultured_VVN these_DD2 so-called_JJ induced_JJ pluripotent_JJ stem_NN1 cells_NN2 (_( iPS_VVZ cells_NN2 )_) in_II a_AT1 mixture_NN1 of_IO chemical_JJ factors_NN2 that_CST triggered_VVD their_APPGE conversion_NN1 into_II liver_NN1 cells_NN2 ,_, which_DDQ had_VHD the_AT appearance_NN1 and_CC functional_JJ properties_NN2 of_IO native_JJ liver_NN1 cells_NN2 ._. 
When_CS the_AT team_NN1 compared_VVD three_MC of_IO the_AT disease-specific_JJ cultures_NN2 with_IW the_AT cells_NN2 generated_VVN from_II the_AT healthy_JJ controls_NN2 ,_, they_PPHS2 discovered_VVD that_CST the_AT diseased_JJ cells_NN2 had_VHD many_DA2 of_IO the_AT key_JJ molecular_JJ defects_NN2 characteristic_NN1 of_IO the_AT original_JJ donors_NN2 '_GE liver_NN1 disorder_NN1 ._. 
An_AT1 example_NN1 comes_VVZ from_II a_AT1 patient_NN1 with_IW familial_JJ hypercholesterolemia_NN1 --_NN1 a_AT1 disease_NN1 that_CST causes_VVZ high_JJ blood_NN1 levels_NN2 of_IO "_" bad_JJ "_" LDL_NN1 (_( low-density_JJ lipoprotein_NN1 )_) cholesterol_NN1 because_CS a_AT1 cellular_JJ defect_NN1 prevents_VVZ the_AT liver_NN1 from_II absorbing_JJ LDL_NN1 ._. 
The_AT new_JJ liver_NN1 cells_NN2 created_VVN from_II this_DD1 patient_NN1 also_RR lacked_VVD the_AT proper_JJ machinery_NN1 to_TO take_VVI up_RP LDL_NN1 ._. 
Cultures_NN2 representing_VVG two_MC other_JJ hereditary_JJ liver_NN1 diseases_NN2 ,_, alpha_NN1 1-antitrypsin_NN1 deficiency_NN1 (_( a_AT1 protein-folding_JJ disorder_NN1 that_CST leads_VVZ to_II cell_NN1 death_NN1 and_CC liver_NN1 failure_NN1 )_) and_CC glycogen_NN1 storage_NN1 disease_NN1 ,_, type_NN1 1a_FO (_( in_II which_DDQ a_AT1 liver_NN1 enzyme_NN1 deficiency_NN1 impairs_VVZ the_AT body_NN1 's_GE glucose_NN1 metabolism_NN1 )_) ,_, also_RR displayed_VVN disorder-specific_JJ abnormalities_NN2 ._. 
The_AT finding_NN1 opens_VVZ up_RP ways_NN2 to_TO investigate_VVI malfunctioning_VVG livers_NN2 ._. 
"_" One_MC1 problem_NN1 in_II liver_NN1 research_NN1 is_VBZ that_CST no_PN121 one_PN122 is_VBZ able_JK to_TO grow_VVI liver_NN1 cells_NN2 in_II the_AT lab_NN1 ,_, "_" says_VVZ the_AT study_NN1 's_GE lead_NN1 author_NN1 ,_, Sheikh_NNB Tamir_NP1 Rashid_NP1 ._. 
That_DD1 inability_NN1 ,_, he_PPHS1 explains_VVZ ,_, has_VHZ made_VVN the_AT mechanisms_NN2 of_IO different_JJ liver_NN1 diseases_NN2 difficult_JJ to_TO understand_VVI ._. 
"_" Now_RT it_PPH1 's_VBZ going_VVGK to_TO be_VBI possible_JJ to_TO ask_VVI at_II the_AT level_NN1 of_IO the_AT individual_NN1 what_DDQ is_VBZ going_VVG on_RP ._. 
We_PPIS2 can_VM then_RT identify_VVI the_AT right_JJ targets_NN2 for_IF therapeutic_JJ drugs_NN2 ,_, "_" he_PPHS1 adds_VVZ ._. 
This_DD1 proof-of-principle_JJ study_NN1 shows_VVZ "_" for_IF the_AT first_MD time_NNT1 ..._... that_DD1 human_NN1 iPS_VVZ cells_NN2 can_VM be_VBI used_VVN to_TO model_VVI a_AT1 diverse_JJ range_NN1 of_IO inherited_JJ diseases_NN2 in_II adult_NN1 cells_NN2 ,_, "_" the_AT authors_NN2 wrote_VVD in_II their_APPGE paper_NN1 ,_, published_VVD online_RR in_II The_AT Journal_NN1 of_IO Clinical_JJ Investigation_NN1 August_NPM1 25_MC ._. 
They_PPHS2 suggested_VVD that_CST their_APPGE approach_NN1 could_VM be_VBI used_VVN to_TO study_VVI other_JJ ,_, more_RGR common_JJ metabolic_JJ disorders_NN2 ._. 
Ludovic_NP1 Vallier_NP1 ,_, one_MC1 of_IO the_AT paper_NN1 's_GE corresponding_JJ authors_NN2 ,_, said_VVD in_II a_AT1 prepared_JJ statement_NN1 :_: "_" Our_APPGE work_NN1 represents_VVZ an_AT1 important_JJ step_NN1 towards_II delivering_VVG the_AT clinical_JJ promises_NN2 of_IO stem_NN1 cells_NN2 ._. "_" 
Rashid_NP1 's_GE ultimate_JJ hope_NN1 is_VBZ that_CST one_MC1 day_NNT1 scientsts_NN2 will_VM be_VBI able_JK to_TO correct_VVI the_AT diseased_JJ cells_NN2 in_II the_AT lab_NN1 and_CC transplant_VV0 them_PPHO2 back_RP into_II the_AT patient_NN1 ,_, but_CCB he_PPHS1 cautions_VVZ that_CST cell-based_JJ therapies_NN2 wo_VM n't_XX be_VBI available_JJ anytime_NNT1 soon_RR ._. 
One_MC1 problem_NN1 with_IW using_VVG iPS_NN2 cells_NN2 therapeutically_RR is_VBZ that_CST the_AT reprogramming_JJ process_NN1 creates_VVZ cells_NN2 prone_JJ to_II forming_VVG tumors_NN2 ._. 
Furthermore_RR ,_, regulations_NN2 stipulate_VV0 that_CST each_DD1 new_JJ cell_NN1 line_NN1 generated_VVN be_VBI independently_RR tested_VVN and_CC validated_VVN for_IF safety_NN1 ,_, making_VVG it_PPH1 impractical_JJ to_TO develop_VVI patient-specific_JJ cells_NN2 for_IF therapies_NN2 ._. 
For_IF now_RT ,_, Rashid_NP1 's_GE group_NN1 has_VHZ a_AT1 list_NN1 of_IO candidate_NN1 therapeutic_JJ drugs_NN2 that_CST they_PPHS2 'll_VM test_VVI in_II their_APPGE new_JJ liver_NN1 disease_NN1 models_NN2 ,_, something_PN1 that_CST may_VM still_RR prove_VVI useful_JJ to_II patients_NN2 ._. 
