PMID: 10837493

Mitsuhashi N, Miki T, Senbongi H, Yokoi N, Yano H, Miyazaki M, Nakajima N, Iwanaga T, Yokoyama Y, Shibata T, Seino S
MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis.
J Biol Chem. 2000 Jun 9;275(23):17536-40., [PubMed]
Sentences
No. Mutations Sentence Comment
66 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:66:309
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:66:319
status: NEW
view ABCB6 p.Lys629Arg details
Analysis of MTABC3 Function-The Walker A-encoding region of MTABC3 cDNA was mutated by a PCR-based method using oligonucleotides bearing mismatched bases at the residues to be mutated (5b18;-CCA TCT GGG GCA GCG AGG AGC ACA ATT TTG-3b18; and 5b18;-CAA AAT TGT GCT CCT CGC TGC CCC AGA TGG-3b18; for G628A and K629R, respectively) in combination with oligonucleotides (5b18;-GTT ACC AGT TAC GTC TTC CT-3b18; and 5b18;-TCT TTG AGA GGG AAG TGG CC-3b18;) (24). Login to comment
67 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:67:309
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:67:319
status: NEW
view ABCB6 p.Lys629Arg details
Analysis of MTABC3 Function-The Walker A-encoding region of MTABC3 cDNA was mutated by a PCR-based method using oligonucleotides bearing mismatched bases at the residues to be mutated (5Ј-CCA TCT GGG GCA GCG AGG AGC ACA ATT TTG-3Ј and 5Ј-CAA AAT TGT GCT CCT CGC TGC CCC AGA TGG-3Ј for G628A and K629R, respectively) in combination with oligonucleotides (5Ј-GTT ACC AGT TAC GTC TTC CT-3Ј and 5Ј-TCT TTG AGA GGG AAG TGG CC-3Ј) (24). Login to comment
127 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:127:111
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:127:118
status: NEW
view ABCB6 p.Lys629Arg details
We then mutated the Walker A motif, GPSGAGKST of MTABC3 to GPSGAARST, and the resultant double mutant, MTABC3 (G628A, K629R), was introduced into atm1-1 mutant cells. Login to comment
128 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:128:111
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:128:118
status: NEW
view ABCB6 p.Lys629Arg details
We then mutated the Walker A motif, GPSGAGKST of MTABC3 to GPSGAARST, and the resultant double mutant, MTABC3 (G628A, K629R), was introduced into atm1-1 mutant cells. Login to comment
138 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:138:88
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:138:95
status: NEW
view ABCB6 p.Lys629Arg details
In addition, only 30% of atm1-1 mutant cells transformed with the double mutant MTABC3 (G628A, K629R) maintained respiratory function. Login to comment
139 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:139:88
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:139:95
status: NEW
view ABCB6 p.Lys629Arg details
In addition, only 30% of atm1-1 mutant cells transformed with the double mutant MTABC3 (G628A, K629R) maintained respiratory function. Login to comment
148 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:148:180
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:148:187
status: NEW
view ABCB6 p.Lys629Arg details
Interestingly, although the iron accumulation is only partially reversed (Fig. 3A), the damage of mitochondrial DNA (Fig. 3B) is almost fully reversed by the double mutant MTABC3 (G628A, K629R) in atm1-1 mutant cells. Login to comment
149 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:149:180
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:149:187
status: NEW
view ABCB6 p.Lys629Arg details
Interestingly, although the iron accumulation is only partially reversed (Fig. 3A), the damage of mitochondrial DNA (Fig. 3B) is almost fully reversed by the double mutant MTABC3 (G628A, K629R) in atm1-1 mutant cells. Login to comment
170 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:170:130
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:170:137
status: NEW
view ABCB6 p.Lys629Arg details
A, iron content of mitochondria isolated from yeast YM13-1c strain transformed with pYES2, ATM1, MTABC3, or MTABC3 double mutant (G628A, K629R) (values are mean afe; S.E.). Login to comment
171 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:171:130
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:171:137
status: NEW
view ABCB6 p.Lys629Arg details
A, iron content of mitochondria isolated from yeast YM13-1c strain transformed with pYES2, ATM1, MTABC3, or MTABC3 double mutant (G628A, K629R) (values are mean Ϯ S.E.). Login to comment
173 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:173:174
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:173:181
status: NEW
view ABCB6 p.Lys629Arg details
C, the rates (%) of cells maintaining mitochondrial respiratory function obtained by transforming pYES2 vector (ǧa;), ATM1 (f), MTABC3 (Ǧc;), or MTABC3 double mutant G628A, K629R (cf;) into atm1-1 mutant cells at the indicated times are shown (mean afe; S.E.). Login to comment
174 ABCB6 p.Gly628Ala
X
ABCB6 p.Gly628Ala 10837493:174:176
status: NEW
view ABCB6 p.Gly628Ala details
ABCB6 p.Lys629Arg
X
ABCB6 p.Lys629Arg 10837493:174:183
status: NEW
view ABCB6 p.Lys629Arg details
C, the rates (%) of cells maintaining mitochondrial respiratory function obtained by transforming pYES2 vector (Ⅺ), ATM1 (f), MTABC3 (⅜), or MTABC3 double mutant G628A, K629R (q) into atm1-1 mutant cells at the indicated times are shown (mean Ϯ S.E.). Login to comment