Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003119100 | SCV003790062 | pathogenic | Adrenoleukodystrophy | 2022-10-17 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the ABCD1 gene has been identified. Loss-of-function variants in ABCD1 are known to be pathogenic (PMID: 11748843). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. This variant has not been reported in the literature in individuals affected with ABCD1-related conditions. For these reasons, this variant has been classified as Pathogenic. |
Labcorp Genetics |
RCV003119101 | SCV003790638 | pathogenic | 3-Methylglutaconic aciduria type 2 | 2022-10-04 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the TAZ gene has been identified. Loss-of-function variants in TAZ are known to be pathogenic (PMID: 16427346, 22382802, 23409742). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. A similar copy number variant has been observed in individual(s) with Barth syndrome (PMID: 19396829). For these reasons, this variant has been classified as Pathogenic. |
Labcorp Genetics |
RCV003119102 | SCV003790643 | pathogenic | X-linked Emery-Dreifuss muscular dystrophy | 2022-10-04 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the EMD gene has been identified. Loss-of-function variants in EMD are known to be pathogenic (PMID: 24365856). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. A similar copy number variant has been observed in individuals with Emery–Dreifuss muscular dystrophy (EDMD) (PMID: 9384614, 10480214). For these reasons, this variant has been classified as Pathogenic. |
Labcorp Genetics |
RCV003109219 | SCV003793205 | pathogenic | Spastic paraplegia | 2022-10-17 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the L1CAM gene has been identified. Loss-of-function variants in L1CAM are known to be pathogenic (PMID: 19846429). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. A similar copy number variant has been observed in individual(s) with X-linked hydrocephalus (PMID: 11968085). For these reasons, this variant has been classified as Pathogenic. |
Labcorp Genetics |
RCV003109218 | SCV003793582 | pathogenic | Heterotopia, periventricular, X-linked dominant; Melnick-Needles syndrome; Oto-palato-digital syndrome, type II; Frontometaphyseal dysplasia | 2022-10-17 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the FLNA gene has been identified. Loss-of-function variants in FLNA are known to be pathogenic (PMID: 16684786, 20730588, 26471271). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. A similar copy number variant has been observed in individual(s) with periventricular nodular heterotopia (PMID: 29334594). For these reasons, this variant has been classified as Pathogenic. |
Labcorp Genetics |
RCV003119103 | SCV003794848 | pathogenic | Creatine transporter deficiency | 2022-10-17 | criteria provided, single submitter | clinical testing | A gross deletion of the genomic region encompassing the full coding sequence of the SLC6A8 gene has been identified. Loss-of-function variants in SLC6A8 are known to be pathogenic (PMID: 22281021). The boundaries of this event are unknown as they extend beyond the assayed region for this gene and therefore may encompass additional genes. A similar copy number variant has been observed in individual(s) with clinical features of creatinine transporter deficiency (PMID: 16601897, 23660394, 24962355). For these reasons, this variant has been classified as Pathogenic. |