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3-methylglutaconic Aciduria Type 2: Disease Bioinformatics

Research of 3-methylglutaconic Aciduria Type 2 has been linked to Cardiomyopathies, Myopathy, Cardiomyopathy, Dilated, Genetic Diseases, X-linked, Heart Failure. The study of 3-methylglutaconic Aciduria Type 2 has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to 3-methylglutaconic Aciduria Type 2 include Transport, Pathogenesis, Electron Transport, Oxidative Phosphorylation, Electron Transport Chain. These pathways complement our catalog of research reagents for the study of 3-methylglutaconic Aciduria Type 2 including antibodies and ELISA kits against TAZ, WWTR1, LDB3, ZNF238, BARTH SYNDROME).

Top Research Reagents

We have 1266 products for the study of 3-methylglutaconic Aciduria Type 2 that can be applied to Chromatin Immunoprecipitation, Chromatin Immunoprecipitation (ChIP), Flow Cytometry, Immunocytochemistry/Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

Deoxyribonuclease I like 1 Antibody (4E8)
H00001774-M02
Western Blot: Deoxyribonuclease I like 1 Antibody (4E8) [H00001774-M02] - Analysis of DNASE1L1 expression in transfected 293T cell line by DNASE1L1 monoclonal antibody (M02), clone 4E8.Lane 1: DNASE1L1 transfected lysate(33.9 KDa).Lane 2: Non-transfected lysate.Sandwich ELISA: Deoxyribonuclease I like 1 Antibody (4E8) [H00001774-M02] - Detection limit for recombinant GST tagged DNASE1L1 is approximately 0.3ng/ml as a capture antibody.

Mouse Monoclonal
Species Human
Applications WB, ELISA, ICC/IF

2 Publications
PGS1 Antibody
NBP1-85022
Immunocytochemistry/Immunofluorescence: PGS1 Antibody [NBP1-85022] - Staining of human cell line A-431 shows localization to plasma membrane, cell junctions & vesicles. Antibody staining is shown in green.Immunohistochemistry-Paraffin: PGS1 Antibody [NBP1-85022] - Staining of human bone marrow shows cytoplasmic positivity in hematopoietic cells.

Rabbit Polyclonal
Species Human
Applications ICC/IF, IHC, IHC-P

LPCAT2 Antibody
NBP1-88921
Western Blot: LPCAT2 Antibody [NBP1-88921] - Analysis in mouse cell line NIH-3T3 and rat cell line NBT-II.Immunocytochemistry/Immunofluorescence: LPCAT2 Antibody [NBP1-88921] - Staining of human cell line A-431 shows localization to endoplasmic reticulum & lipid droplets.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

     2 Reviews

5 Publications
LPCAT1 Antibody
NBP1-88923
Immunohistochemistry-Paraffin: LPCAT1 Antibody [NBP1-88923] - Analysis in human lung and liver tissues. Corresponding LPCAT1 RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: LPCAT1 Antibody [NBP1-88923] - Staining of human cerebral cortex, kidney, liver and lung using Anti-LPCAT1 antibody NBP1-88923 (A) shows similar protein distribution across tissues to independent antibody NBP1-88922 (B).

Rabbit Polyclonal
Species Human, Mouse
Applications WB, ICC/IF, IHC

     1 Review

2 Publications
Dystrophin Antibody
NBP1-89953
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining in human skeletal muscle and tonsil tissues . Corresponding DMD RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human heart muscle shows strong membranous positivity in myocytes.

Rabbit Polyclonal
Species Human, Mouse
Applications ICC/IF, IHC, IHC-P

4 Publications
LDB3 Antibody (3C8)
H00011155-M06
Western Blot: LDB3 Antibody (3C8) [H00011155-M06] - Analysis of LDB3 expression in A-431 (Cat # L015V1).Immunocytochemistry/Immunofluorescence: LDB3 Antibody (3C8) [H00011155-M06] - Analysis of monoclonal antibody to LDB3 on A-431 cell. Antibody concentration 10 ug/ml

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ELISA, ICC/IF

5 Publications
MTSS1 Antibody
NBP2-24716
Western Blot: MTSS1 Antibody [NBP2-24716] - Analysis of MTSS1 in human testis lysate in the 1) absence and 2) presence of immunizing peptide, 3) mouse and 4) rat testis lysate using NBP2-24716 at 2 ug/ml, 4 ug/ml and 2 ug/ml, respectively.Immunohistochemistry-Paraffin: MTSS1 Antibody [NBP2-24716] - Analysis of human colon using this antibody at 10 ug/ml.

Rabbit Polyclonal
Species Human, Mouse, Primate
Applications WB, ICC/IF, IHC

1 Publication
Lamin A + C Antibody (4C4)
NBP2-25151
Western Blot: Lamin A + C Antibody (4C4) [NBP2-25151] - Analysis of different cell lysates using mouse mAb to lamin A/C, NBP2-25151, dilution 1:1,000 in green: [1] protein standard (red), [2] HeLa, [3] HEK293 [4] C6, and [5] NIH-3T3 cell lysates. Two strong bands at 74 and 65kDa correspond to the lamin A and lamin C proteins respectively, detected only in the cells of human origin. NBP2-25151 antibody failed to recognize rat or mouse proteins.Immunocytochemistry/Immunofluorescence: Lamin A + C Antibody (4C4) [NBP2-25151] - Analysis of HeLa cells stained with mouse mAb to lamin A/C, NBP2-25151, dilution 1:2,000 in red, and costained with rabbit pAb to HSP60, dilution 1:5,000, in green. The blue is Hoechst staining of nuclear DNA. NBP2-25151 antibody specifically labels the nuclear lamina, while the HSP60 antibody reveals protein expressed in mitochondria.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF

2 Publications
TAZ/WWTR1 Antibody - BSA Free
NB110-58359
Western Blot: TAZ/WWTR1 Antibody [NB110-58359] - Detection of TAZ on HEK 293 cell lysate using NB110-58359.Immunocytochemistry/Immunofluorescence: TAZ/WWTR1 Antibody [NB110-58359] - Detection of TAZ (Green) in HepG2 cells using NB110-58359. Nuclei (Blue) are counterstained with Hoecsht 33258.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, ChIP

     1 Review

18 Publications
Desmin Antibody
AF3844
Desmin was detected in immersion fixed paraffin-embedded sections of human heart using 15 µg/mL Human Desmin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3844) overnight at 4 °C. Tissue was stained with the Anti-Goat HRP-DAB Cell & Tissue Staining Kit (brown; Catalog # <A class=Desmin was detected in immersion fixed frozen sections of mouse skeletal muscle using Human Desmin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3844) at 10 µg/mL overnight at 4 °C. Tissue was stained using the NorthernLights™ 557-conjugated Anti-Goat IgG Secondary Antibody (red; Catalog # <A class=NoLineLink href=

Goat Polyclonal
Species Human, Mouse
Applications IHC

     2 Reviews

16 Publications
Tafazzin/TAZ Antibody (1B10)
H00006901-M12
Western Blot: Tafazzin/TAZ Antibody (1B10) [H00006901-M12] - Taz protein expression is higher in the soleus compared with plantaris and is upregulated in response to both overload (OVL) and unload (UNL) stimuli. (a) Taz protein expression (~27 kDa) in the sham plantaris and soleus muscles. (b) Taz protein in the OVL plantaris compared with sham. (c) Taz protein in the UNL soleus compared with sham. *p <= 0.05 using a Student's t-test (a, two-tailed) or paired t-test (b and c, two-tailed), n = 6 per group. Please refer to Fig. S4 for full length Western blots. Image collected and cropped by CiteAb from the following publication (https://pubmed.ncbi.nlm.nih.gov/28515468) licensed under a CC-BY license.Western Blot: Tafazzin/TAZ Antibody (1B10) [H00006901-M12] - Analysis of TAZ expression in PC-12 (Cat # L012V1).

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, ELISA, ICC/IF

     1 Review

6 Publications
DMPK Antibody (713931)
MAB6967
Western blot shows lysates of human heart tissue. PVDF membrane was probed with 2 µg/mL of Mouse Anti-Human DMPK Monoclonal Antibody (Catalog # MAB6967) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # <A class=NoLineLink href=

Mouse Monoclonal
Species Human
Applications WB

BID Antibody [Unconjugated]
AF860
Western blot shows Jurkat human acute T cell leukemia cell line treated with apoptosis inducer anti-Fas for the indicated times. PVDF membrane was probed with 1 µg/mL Goat Anti-Human/Mouse BID Antigen Affinity-purified Polyclonal Antibody (Catalog # AF860) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <A class=NoLineLink href= Western blot shows lysates of SVEC4‑10 mouse vascular endothelial cell line and Jurkat human acute T cell leukemia cell line. PVDF membrane was probed with 1 µg/mL of Goat Anti-Human/Mouse BID Antigen Affinity-purified Polyclonal Antibody (Catalog # AF860) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # <a class=

Goat Polyclonal
Species Human, Mouse
Applications WB, Simple Western, IP

     1 Review

22 Publications
Cytochrome c Antibody (7H8.2C12) - BSA Free
NB100-56503
Western Blot: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - Metformin activates apoptotic cell death; Right panel: MCF-7 cells were kept in the presence or absence of metformin for 20 days and then processed to obtain mitochondrial or whole cellular extracts. BAX and CYCS expression levels were determined by Western blot as indicated under Material and Methods. Densitometric analysis of the gels was performed as indicated under Material and Methods. PHB and CDK4 were used as purity and loading controls. Image collected and cropped by CiteAb from the following publication (http://www.mdpi.com/2073-4409/8/1/49), licensed under a CC-BY license.Immunocytochemistry/Immunofluorescence: Cytochrome c Antibody (7H8.2C12) [NB100-56503] - p73 was detected in immersion fixed Hela human cell line using NB100-56503 at 25 ug/ml for 3 hours at room temperature. Cells were stained using the NorthernLights(TM) 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # NL007) and counterstained with DAPI (blue). Staining was observed in the cytoplasm and mitochondria.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, Simple Western, Flow

60 Publications
Dystrobrevin alpha Antibody (OTI1B2) - Azide and BSA Free
NBP2-70591
Western Blot: Dystrobrevin alpha Antibody (OTI1B2) - Azide and BSA Free [NBP2-70591] - HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY Dystrobrevin alpha (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-DysImmunocytochemistry/Immunofluorescence: Dystrobrevin alpha Antibody (OTI1B2) - Azide and BSA Free [NBP2-70591] - Staining of COS7 cells transiently transfected by pCMV6-ENTRY Dystrobrevin alpha.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

ZNF238 Antibody
NBP2-82381
Western Blot: ZNF238 Antibody [NBP2-82381] - Host: Rabbit. Target Name: ZNF238. Sample Type: Human Fetal Stomach. Antibody Dilution: 1.0ug/mlWestern Blot: ZNF238 Antibody [NBP2-82381] - WB Suggested Anti-ZNF238 Antibody Titration: 0.2-1 ug/ml. ELISA Titer: 1:312500. Positive Control: Human Pancreas

Rabbit Polyclonal
Species Human
Applications WB

LPCAT3 Antibody - Azide and BSA Free
NBP3-04752
Western Blot: LPCAT3 Antibody [NBP3-04752] - Analysis of extracts of various cell lines, using LPCAT3 antibody (NBP3-04752) at 1:500 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit. Exposure time: 180s.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB

1 Publication

Related Genes

3-methylglutaconic Aciduria Type 2 has been researched against:

Related Pathways

3-methylglutaconic Aciduria Type 2 has been linked to:

Related Diseases

3-methylglutaconic Aciduria Type 2 has been studied in relation to diseases such as:

Related PTMs

3-methylglutaconic Aciduria Type 2 has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

3-methylglutaconic Aciduria Type 2 is also known as Barth Syndrome, Cardioskeletal Myopathy With Neutropenia And Abnormal Mitochondria, Syndrome, Barth.