HPA042158

antibody from Atlas Antibodies

Targeting: GTPBP3 FLJ14700, GTPBG3, MSS1, MTGP1, THDF1

Western blot (Data presented on provider website)

Immunocytochemistry (Supportive data in Antibodypedia)

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

HPA042158

Provider

Atlas Antibodies

Proper citation

Atlas Antibodies Cat#HPA042158, RRID:AB_10964400

Product name

Anti-GTPBP3

Antibody type

Polyclonal

Description

Polyclonal Antibody against Human GTPBP3, Gene description: GTP binding protein 3 (mitochondrial), Alternative Gene Names: FLJ14700, GTPBG3, MSS1, MTGP1, THDF1, Validated applications: ICC, IHC, WB, Uniprot ID: Q969Y2, Storage: Store at +4°C for short term storage. Long time storage is recommended at -20°C.

Reactivity

Human

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

100 µl

Concentration

0.2 mg/ml

Storage

Store at +4°C for short term storage. Long time storage is recommended at -20°C.

Handling

The antibody solution should be gently mixed before use.

References

Submitted references

Restoration of mitochondrial function through activation of hypomodified tRNAs with pathogenic mutations associated with mitochondrial diseases

Tomoda E, Nagao A, Shirai Y, Asano K, Suzuki T, Battersby B, Suzuki T
Nucleic Acids Research 2023;51(14):7563-7579

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Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism

Chen D, Zhang Z, Chen C, Yao S, Yang Q, Li F, He X, Ai C, Wang M, Guan M
Nucleic Acids Research 2019;47(10):5341-5355

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Mutations in GTPBP3 Cause a Mitochondrial Translation Defect Associated with Hypertrophic Cardiomyopathy, Lactic Acidosis, and Encephalopathy

Kopajtich R, Nicholls T, Rorbach J, Metodiev M, Freisinger P, Mandel H, Vanlander A, Ghezzi D, Carrozzo R, Taylor R, Marquard K, Murayama K, Wieland T, Schwarzmayr T, Mayr J, Pearce S, Powell C, Saada A, Ohtake A, Invernizzi F, Lamantea E, Sommerville E, Pyle A, Chinnery P, Crushell E, Okazaki Y, Kohda M, Kishita Y, Tokuzawa Y, Assouline Z, Rio M, Feillet F, Mousson de Camaret B, Chretien D, Munnich A, Menten B, Sante T, Smet J, Régal L, Lorber A, Khoury A, Zeviani M, Strom T, Meitinger T, Bertini E, Van Coster R, Klopstock T, Rötig A, Haack T, Minczuk M, Prokisch H
The American Journal of Human Genetics 2014;95(6):708-720

Immunocytochemistry

Supportive validation

Submitted by

Atlas Antibodies (provider)

Main image

Experimental details

Immunofluorescent staining of human cell line U-251 MG shows localization to mitochondria.

Sample type

Human