mtx2 (Proteintech)

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Proteintech mtx2

Clinical manifestations and mutations of gene <t>MTX2</t> in probands. (A) Photograph of patient 1. The boy showed specific facial features (sparse hair; frontal bossing; exophthalmos; prominent ear; thick lip; crowding teeth; micrognathia). (B) Renal ultrasound of patient 1. (C) Cranial CT in patient 1. (D) Echocardiography of patient 1. (E) Representative images of PASM staining in kidney of patient 2. yellow arrows, “pseudo double track”, red arrows, thickening of basement membrane, scale bar: 25 μm. (F) Masson staining in kidney of patient 2. red arrows, deposition of fuchsinophilic protein in mesangial area and basement membrane, scale bar: 25 μm. (G) Transmission EM images in kidney of patient 2. red arrows, electronic dense deposits in subendothelial, mesangial areas and subepithelial, scale bar: 5 μm. (H) MTX2 mutations in families. The compound heterozygous mutation including c.378+1 (IVS6) G>A mutation and c.95 (exon3) delA mutation in patient 1 and the c.500 (exon8) _c.501 (exon8) insT homozygous mutation in patient 2 was identified by WES. Circles indicate females; squares indicate males; solid indicate patients; arrows indicate patients.

Mtx2, supplied by Proteintech, used in various techniques. Bioz Stars score: 93/100, based on 4 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/product/immunohistochemistry+microarray+based+expression+data/MTX2+Antibody/pmc10797693-185-8-10
Average 93 stars, based on 4 article reviews

mtx2 - by Bioz Stars, 2026-10

93/100 stars

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1) Product Images from "Loss of MTX2 causes mitochondrial dysfunction, podocyte injury, nephrotic proteinuria and glomerulopathy in mice and patients"

Article Title: Loss of MTX2 causes mitochondrial dysfunction, podocyte injury, nephrotic proteinuria and glomerulopathy in mice and patients

Journal: International Journal of Biological Sciences

doi: 10.7150/ijbs.89916

Clinical manifestations and mutations of gene MTX2 in probands. (A) Photograph of patient 1. The boy showed specific facial features (sparse hair; frontal bossing; exophthalmos; prominent ear; thick lip; crowding teeth; micrognathia). (B) Renal ultrasound of patient 1. (C) Cranial CT in patient 1. (D) Echocardiography of patient 1. (E) Representative images of PASM staining in kidney of patient 2. yellow arrows, “pseudo double track”, red arrows, thickening of basement membrane, scale bar: 25 μm. (F) Masson staining in kidney of patient 2. red arrows, deposition of fuchsinophilic protein in mesangial area and basement membrane, scale bar: 25 μm. (G) Transmission EM images in kidney of patient 2. red arrows, electronic dense deposits in subendothelial, mesangial areas and subepithelial, scale bar: 5 μm. (H) MTX2 mutations in families. The compound heterozygous mutation including c.378+1 (IVS6) G>A mutation and c.95 (exon3) delA mutation in patient 1 and the c.500 (exon8) _c.501 (exon8) insT homozygous mutation in patient 2 was identified by WES. Circles indicate females; squares indicate males; solid indicate patients; arrows indicate patients.

Figure Legend Snippet: Clinical manifestations and mutations of gene MTX2 in probands. (A) Photograph of patient 1. The boy showed specific facial features (sparse hair; frontal bossing; exophthalmos; prominent ear; thick lip; crowding teeth; micrognathia). (B) Renal ultrasound of patient 1. (C) Cranial CT in patient 1. (D) Echocardiography of patient 1. (E) Representative images of PASM staining in kidney of patient 2. yellow arrows, “pseudo double track”, red arrows, thickening of basement membrane, scale bar: 25 μm. (F) Masson staining in kidney of patient 2. red arrows, deposition of fuchsinophilic protein in mesangial area and basement membrane, scale bar: 25 μm. (G) Transmission EM images in kidney of patient 2. red arrows, electronic dense deposits in subendothelial, mesangial areas and subepithelial, scale bar: 5 μm. (H) MTX2 mutations in families. The compound heterozygous mutation including c.378+1 (IVS6) G>A mutation and c.95 (exon3) delA mutation in patient 1 and the c.500 (exon8) _c.501 (exon8) insT homozygous mutation in patient 2 was identified by WES. Circles indicate females; squares indicate males; solid indicate patients; arrows indicate patients.

Techniques Used: Staining, Membrane, Transmission Assay, Mutagenesis

Renal phenotype in Pod-Mtx2-KO mice. (A) Western blots and quantification analysis, showing reduced MTX2 expression in primary glomerular cells from Pod-Mtx2-KO mice compared with control mice (n = 3). (B) The summary data on body weights of mice (n = 10). (C) The summary data on kidney weights of mice (n = 10). (D) Representative images of kidney from Pod-Mtx2-KO and control mice at 3, 6, and 9 mo of age. (E) 24 h urinary albumin quantification of Pod-Mtx2-KO and control mice at 8, 12, 16 weeks of age (n = 10). (F) Coomassie blue staining of urine from Pod-Mtx2-KO and control mice at 12 weeks of age as well as standard BSA with different concentrations. (G) Representative images showing F-actin cytoskeleton stained with FITC labeled phalloidin in primary podocytes isolated from Pod-Mtx2-KO and control mice at 8 weeks of age, scale bars: 50 μm. WT1, a specific marker protein of podocytes to identify isolated primary glomerular podocytes. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Figure Legend Snippet: Renal phenotype in Pod-Mtx2-KO mice. (A) Western blots and quantification analysis, showing reduced MTX2 expression in primary glomerular cells from Pod-Mtx2-KO mice compared with control mice (n = 3). (B) The summary data on body weights of mice (n = 10). (C) The summary data on kidney weights of mice (n = 10). (D) Representative images of kidney from Pod-Mtx2-KO and control mice at 3, 6, and 9 mo of age. (E) 24 h urinary albumin quantification of Pod-Mtx2-KO and control mice at 8, 12, 16 weeks of age (n = 10). (F) Coomassie blue staining of urine from Pod-Mtx2-KO and control mice at 12 weeks of age as well as standard BSA with different concentrations. (G) Representative images showing F-actin cytoskeleton stained with FITC labeled phalloidin in primary podocytes isolated from Pod-Mtx2-KO and control mice at 8 weeks of age, scale bars: 50 μm. WT1, a specific marker protein of podocytes to identify isolated primary glomerular podocytes. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Techniques Used: Western Blot, Expressing, Control, Staining, Labeling, Isolation, Marker

Renal histology and ultrastructural morphology in Pod-Mtx2-KO mice. (A) HE staining of glomerular mesangial hyperplasia in different degrees in Pod-Mtx2-KO mice kidneys during the 40 week follow-up (n = 6), yellow arrows, mesangial cells, scale bar: 50 μm. (B) Images of HE staining demonstrating no significant difference in renal tubules between Pod-Mtx2-KO and control mice during the 40 week follow-up (n = 6), scale bar: 100 μm. (C) Transmission EM images, showing fusion of podocyte foot processes, abnormal basement membrane and mesangial matrix proliferation in glomerulus of Pod-Mtx2-KO mice at different ages (n = 6), scale bar: 5 μm. (D) Images of scanning EM showing abnormal podocyte foot process architecture in Pod-Mtx2-KO mice at different ages (n = 6), scale bar: 2 μm. (E) Images of transmission EM illustrating mitochondrial structural abnormalities and increased autophagosomes in podocytes of Pod-Mtx2-KO mice during the 40 week follow-up (n = 6), yellow arrows, abnormal structure of mitochondria; red asterisk, autophagosomes, scale bar: 250 nm.

Figure Legend Snippet: Renal histology and ultrastructural morphology in Pod-Mtx2-KO mice. (A) HE staining of glomerular mesangial hyperplasia in different degrees in Pod-Mtx2-KO mice kidneys during the 40 week follow-up (n = 6), yellow arrows, mesangial cells, scale bar: 50 μm. (B) Images of HE staining demonstrating no significant difference in renal tubules between Pod-Mtx2-KO and control mice during the 40 week follow-up (n = 6), scale bar: 100 μm. (C) Transmission EM images, showing fusion of podocyte foot processes, abnormal basement membrane and mesangial matrix proliferation in glomerulus of Pod-Mtx2-KO mice at different ages (n = 6), scale bar: 5 μm. (D) Images of scanning EM showing abnormal podocyte foot process architecture in Pod-Mtx2-KO mice at different ages (n = 6), scale bar: 2 μm. (E) Images of transmission EM illustrating mitochondrial structural abnormalities and increased autophagosomes in podocytes of Pod-Mtx2-KO mice during the 40 week follow-up (n = 6), yellow arrows, abnormal structure of mitochondria; red asterisk, autophagosomes, scale bar: 250 nm.

Techniques Used: Staining, Control, Transmission Assay, Membrane

Podocyte function in MPC5 with or without MTX2 and apoptosis protein microarray assay. (A) Quantification of adherent podocytes after podocytes were seeded on plates coated with collagen for 1 h. (B) Representative images and quantification of migrating podocytes after migration through transwell chamber membranes for 24 h, scale bar: 250 μm. (C) Quantification of the mean fluorescence intensity of FITC-transferrin engulfed by podocytes for 5 h. (D) Quantification of attached podocytes with or without puromycin aminonucleoside (PAN) treatment. (E) F-actin cytoskeleton stained with FITC labeled phalloidin in podocytes, scale bar: 50 μm. (F) Podocyte apoptosis measured by propidium iodide (PI) and FITC-conjugated Annexin V. (G) Representative images of Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis by mouse apoptosis protein microarray performed on MTX2-KD and control podocytes. (H) Representative heatmap of mouse apoptosis associated proteins by mouse apoptosis protein microarray. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Figure Legend Snippet: Podocyte function in MPC5 with or without MTX2 and apoptosis protein microarray assay. (A) Quantification of adherent podocytes after podocytes were seeded on plates coated with collagen for 1 h. (B) Representative images and quantification of migrating podocytes after migration through transwell chamber membranes for 24 h, scale bar: 250 μm. (C) Quantification of the mean fluorescence intensity of FITC-transferrin engulfed by podocytes for 5 h. (D) Quantification of attached podocytes with or without puromycin aminonucleoside (PAN) treatment. (E) F-actin cytoskeleton stained with FITC labeled phalloidin in podocytes, scale bar: 50 μm. (F) Podocyte apoptosis measured by propidium iodide (PI) and FITC-conjugated Annexin V. (G) Representative images of Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis by mouse apoptosis protein microarray performed on MTX2-KD and control podocytes. (H) Representative heatmap of mouse apoptosis associated proteins by mouse apoptosis protein microarray. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Techniques Used: Microarray, Migration, Fluorescence, Staining, Labeling, Control

Mitochondrial function in MPC5 with or without MTX2. (A) Western blots and quantifications analysis of some subunits in respiratory chain complex. TOM20 as the loading control. (B) Quantifications of enzyme activities of oxidative respiratory chains (complex I, III and IV) in MTX2-KD and control podocytes. (C) Quantifications of whole cell ATP production in MPC5 with MTX2 knockdown. (D) Measurement of MMP using the fluorescence probe JC-1 assay kit in MTX2-KD and control podocytes. (E) The OCR curves of podocytes with and without MTX2, stimulated with different inhibitors (oligomycin (1.5 μM), FCCP (2 μM), Retenone /Antimycin (0.5 μM)) at specific time points and quantification of the OCRs. (F) The OCR curves of podocytes with MTX2 overexpressing, compared with the control. (G) Mitochondrial ROS assayed by flow cytometry using the fluorescent probe DCFH-DA in MTX2-KD and control podocytes. (H) Mitochondrial ROS measurement with MTX2 overexpression, compared to the controls. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Figure Legend Snippet: Mitochondrial function in MPC5 with or without MTX2. (A) Western blots and quantifications analysis of some subunits in respiratory chain complex. TOM20 as the loading control. (B) Quantifications of enzyme activities of oxidative respiratory chains (complex I, III and IV) in MTX2-KD and control podocytes. (C) Quantifications of whole cell ATP production in MPC5 with MTX2 knockdown. (D) Measurement of MMP using the fluorescence probe JC-1 assay kit in MTX2-KD and control podocytes. (E) The OCR curves of podocytes with and without MTX2, stimulated with different inhibitors (oligomycin (1.5 μM), FCCP (2 μM), Retenone /Antimycin (0.5 μM)) at specific time points and quantification of the OCRs. (F) The OCR curves of podocytes with MTX2 overexpressing, compared with the control. (G) Mitochondrial ROS assayed by flow cytometry using the fluorescent probe DCFH-DA in MTX2-KD and control podocytes. (H) Mitochondrial ROS measurement with MTX2 overexpression, compared to the controls. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Techniques Used: Western Blot, Control, Knockdown, Fluorescence, Flow Cytometry, Over Expression

Alterations in mitochondrial dynamics, structure, membrane proteins and the Sam50-CHCHD3-Mitofilin axis. (A) Western blots and quantification analysis of mitochondrial membrane proteins (MTX1, VDAC, TOM40) in MTX2-KD and control podocytes. (B) Western blots and quantification analysis of the Sam50-CHCHD3-Mitofilin axis in MTX2-KD and control podocytes. (C) Images of mitochondrial ultrastructure by transmission EM in MTX2-KD podocytes. yellow arrows, abnormal structure of mitochondria; red asterisk, autophagosomes, scale bars: 2 μm. (D) Western blots assay in MTX2-KD podocytes compared to controls, observing decreased levels of mitochondrial fusion proteins MFN2 and OPA1 (including OPA1-L and OPA1-S) and increased levels of mitochondrial fission proteins DRP1 and FIS1. (E) Western blots and quantification analysis showing increased LC3-II/I ratio in MTX2-KD podocytes. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Figure Legend Snippet: Alterations in mitochondrial dynamics, structure, membrane proteins and the Sam50-CHCHD3-Mitofilin axis. (A) Western blots and quantification analysis of mitochondrial membrane proteins (MTX1, VDAC, TOM40) in MTX2-KD and control podocytes. (B) Western blots and quantification analysis of the Sam50-CHCHD3-Mitofilin axis in MTX2-KD and control podocytes. (C) Images of mitochondrial ultrastructure by transmission EM in MTX2-KD podocytes. yellow arrows, abnormal structure of mitochondria; red asterisk, autophagosomes, scale bars: 2 μm. (D) Western blots assay in MTX2-KD podocytes compared to controls, observing decreased levels of mitochondrial fusion proteins MFN2 and OPA1 (including OPA1-L and OPA1-S) and increased levels of mitochondrial fission proteins DRP1 and FIS1. (E) Western blots and quantification analysis showing increased LC3-II/I ratio in MTX2-KD podocytes. Data was shown as the mean ± SD of triplicates at least. *, P < 0.05, **, P < 0.01, ***, P < 0.001.

Techniques Used: Membrane, Western Blot, Control, Transmission Assay

other:

Article Title: Sam50-Mic19-Mic60 axis Determines Mitochondrial Cristae Architecture by Mediating Mitochondrial Outer and Inner Membrane Contact
Article Snippet: Antibodies were used in this study: anti-Metaxin-2, anti-Prohibitin-2, anti-Mic60, anti-Yme1L and anti-SDHA were purchased from Proteintech; anti-Mfn1, anti-Mfn2 and anti-Sam50 were from Abcam; anti-Metaxin-1, anti-Mic19 and anti-HSP60 were from Abclonal; anti-OMA1 was from Santa Cruz Biotechnology; anti-Mic10 was from Origene; anti-Drp1 and anti-OPA1 were purchased from BD Biosciences.

Western Blot:

Article Title: Disruption of axonal transport in Parkinson’s disease: the role of pathological α-Syn and AMPK/p38 MAPK signaling
Article Snippet: .. The antibodies used in western blot were as follows: KIF5B (Abcam, ab5629), KLC1 (Abcam, ab174273), KLC2 (Proteintech, 17668), Miro1 (ABclonal, A5838), MTX1 (Proteintech, 11529), MTX2 (Proteintech, 11610), Dynactin (ABclonal, A1783), DIC (Abcam, ab171964), snapin (Proteintech, 10055), TrkB (CST, Q16620 ), BDNF (Proteintech, 66292), Erk1/2 (Proteintech, 66192), pErk1/2 (ABclonal, AP0974), Rab7 (ABclonal, A12784), PIKE (Proteintech, 14690), AMPK (CST, 5831S), p-AMPK (CST, 2535S), p38 (CST, 8690S), p-p38 (CST, 4511S), α-synuclein (Abcam, ab212184), α-synuclein pSer129 (Abcam, ab51253), TH (Abcam, ab75875), DAT (Proteintech, 22525), MK2 (ABclonal, A10668), CREB (ABclonal, A11989), p-CREB (ABclonal, AP0903). ..

Article Title: Loss of MTX2 causes mitochondrial dysfunction, podocyte injury, nephrotic proteinuria and glomerulopathy in mice and patients
Article Snippet: Signals were detected with enhanced chemiluminescence reagents (#32209; Thermo Fisher). .. Antibodies used in this work were as follows: MTX2 (11610-1-AP, Proteintech) diluted at 1:250 for immunohistochemistry (IHC) and 1:600 for western blotting (WB); MTX2 (sc-514231, Santa Cruz) diluted at 1:250 for immunofluorescence (IF); synaptopodin (21064-1-AP, Proteintech) diluted at 1:400 for IF; Alexa Fluor 594 donkey anti-rabbit IgG (H+L) secondary antibody (A21207; Life Technologies) diluted at 1:500 for IF; Alexa Fluor 488 Goat anti-mouse IgG, IgM (H+L) secondary antibody (A-10680, Invitrogen) diluted at 1:10000 for IF; WT1 (DF6331, Affinity Biosciences) diluted at 1:250 for IF; ND2 (A17968, ABclonal) diluted at 1:1000 for WB; ND4 (A9941, ABclonal) diluted at 1:1000 for WB; ND5 (A17972, ABclonal) diluted at 1:1000 for WB; CYTB (A17966, ABclonal) diluted at 1:1000 for WB; MTCO2 (55070-1-AP, Proteintech) diluted at 1:2000 for WB; NDUFA9 (A3196, ABclonal) diluted at 1:1000 for WB; NDUFS1 (12444-1-AP, Proteintech) diluted at 1:5000 for WB; SDHB (10620-1-AP, Proteintech) diluted at 1: 20000 for WB; UQCRC1 (21705-1-AP, Proteintech) diluted at 1:4000 for WB; ATPB (17247-1-AP, Proteintech) diluted at 1:3000 for WB; TOM20 (A19403, ABclonal) diluted at 1:5000 for WB; MFN2 (12186-1-AP, Proteintech) diluted at 1: 5000 for WB; OPA1 (27733-1-AP, Proteintech) diluted at 1:2000 for WB; DRP1 (12957-1-AP, Proteintech) diluted at 1:5000 for WB; FIS1 (10956-1-AP, Proteintech) diluted at 1:1500 for WB; LC3B (ab192890, Abcam) diluted at 1:2000 for WB; Metaxin 1 (sc-135989, Santa Cruz) diluted at 1:500 for WB; VDAC1/2 (10866-1-AP, Proteintech) diluted at 1:1500 for WB; TOMM40 (18409-1-AP, Proteintech) diluted at 1:8000 for WB; Sam50 (28679-1-AP, Proteintech) diluted at 1:8000 for WB; Mitofilin (10179-1-AP, Proteintech) diluted at 1:1000 for WB; CHCHD3 (25625-1-AP, Proteintech) diluted at 1:8000 for WB. ..

Article Title: Disruption of axonal transport in Parkinson's disease: the role of pathological α-Syn and AMPK/p38 MAPK signaling.
Article Snippet: .. The antibodies used in western blot were as follows: KIF5B (Abcam, ab5629), KLC1 (Abcam, ab174273), KLC2 (Proteintech, 17668), Miro1 (ABclonal, A5838), MTX1 (Proteintech, 11529), MTX2 (Proteintech, 11610), Dynactin (ABclonal, A1783), DIC (Abcam, ab171964), snapin (Proteintech, 10055), TrkB (CST, Q16620), BDNF (Proteintech, 66292), Erk1/2 (Proteintech, 66192), pErk1/2 (ABclonal, AP0974), Rab7 (ABclonal, A12784), PIKE (Proteintech, 14690), AMPK (CST, 5831S), p-AMPK (CST, 2535S), p38 (CST, 8690S), p-p38 (CST, 4511S), α-synuclein (Abcam, ab212184), α-synuclein pSer129 (Abcam, ab51253), TH (Abcam, ab75875), DAT (Proteintech, 22525), MK2 (ABclonal, A10668), CREB (ABclonal, A11989), p-CREB (ABclonal, AP0903). ..

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mtx2 (Proteintech)

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1) Product Images from "Loss of MTX2 causes mitochondrial dysfunction, podocyte injury, nephrotic proteinuria and glomerulopathy in mice and patients"

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